A system of practical medicine. By American authors. Vol. 2 (part 2 of 8)

A system of practical medicine. By American authors. Vol. 2 (part 2 of 8)

In some cases the ulceration begins in the petechiæ at the hair-roots, and a number of these, running together, form a large ulcer. The destruction of tissue by ulceration is disposed to spread more widely and deeply, and is often of a most intractable character. Old cicatrices {179} are the first tissues in these cases to take on the ulcerative action. Certain muscles, chiefly those of the legs, and notably the gastrocnemii, the abdominal and pectoral muscles, the psoas magnus, and pterygoids, may become the seat of fibrinous extravasations, which finally change, by lapse of time, into hard, firm tumors, impairing the functions of those parts and leading to contractions of the limbs.

The symptoms in certain epidemics of extraordinary severity have displayed alterations in still deeper structures. Effusions occur between the periosteum and the bone, forming painful, hard, and resisting nodes of varying dimensions, especially along the course of the tibiæ, upon the scapulæ, and upon the maxillæ. In young persons the epiphyses are separated from the shaft of the long bones, and in other cases the ribs become necrosed and disarticulated from the sternum, producing a creaking noise during respiratory movements, as related by Poupart.[15] This occurs mostly on one side and about the middle of the series, yet it has been noted to occur on both sides, so that the sternum and attached cartilages, deprived of support, were perceptibly sunken. Oserctzkowski[16] reports two fatal cases of scurvy attended with spontaneous fracture of the ribs. There was extravasation into and beneath the periosteum, and subsequent destruction of the continuity of the bone. In one case the ribs on both sides were affected, so that the anterior wall of the thorax sunk in and embarrassed the respiration, which was chiefly maintained by the diaphragm. Inflammation of the lungs succeeded, and the patient died in agony.

[Footnote 15: Mémoires de l'Académie des Sciences, p. 237, 1699, and Philosophical Transactions, vol. xv.]

[Footnote 16: Wratsel, No. 51, 1881.]

Recently-repaired fractures have been known to recur under the influence of scurvy from the destruction of the callus.[17]

[Footnote 17: Anson's Voyage Around the World, edited by Walter.]

The articulations as well as the bones in very severe cases of scurvy present evidences of disease, consisting in periostitic effusions which involve the surrounding soft parts, producing impairment of motion, enlargement, and false ankylosis, and even destroying the normal anatomical relation of the osseous surfaces, so as to determine deformities. These changes are usually attended with severe pain, and most commonly occur in the ankle-, knee-, shoulder-, and hip-joints, and disappear tardily, requiring perhaps months for their recession, if indeed this takes place at all.

The symptoms manifested by the circulatory organs are prominent from an early period of the disease. The pulsations of the heart are slower, feebler, irregular, and often intermittent; its impulse is decreased or becomes quite imperceptible; and when the associated anæmia has progressed to a certain extent a systolic murmur may be audible. The arterial and venous channels are of diminished calibre; the pulse becomes soft, of less volume, and tardier; and a venous murmur may sometimes be heard in the cervical veins. The remarkable nutritive changes in the capillary walls in part account for the numerous hemorrhages which occur both by rhexis and diapedesis. The most frequent is epistaxis; the slightest blows, sneezing, or blowing the nose will often determine it, or it may occur spontaneously, and in severer cases with such profuseness as to threaten impending dissolution, requiring nothing less than timeous introduction of the tampon to rescue the victim. Hemorrhage from the {180} lungs is of rare occurrence, and when it does happen is rather indicative of pre-existing pulmonary disease, such as phthisis, or of the approach of a complication, such as infarction or gangrene, than a constituent feature of scurvy. Hæmatemesis is less uncommon, but is by no means frequent; the blood ejected from the stomach is usually small in quantity, but in isolated examples the bleeding is profuse, producing great exhaustion and a sense of cardiac depression which preludes speedy death. Hemorrhage from the bowels is also an ill-omened feature, completely blanching the patient and presaging early exhaustion and death. Blood may also appear as a product of a complicating dysentery which determines abundant, offensive discharges that may run on for several weeks before the patient is finally exhausted. Hæmaturia sometimes occurs, especially in broken-down and cachectic subjects and in an advanced stage of scurvy. All of these forms of hemorrhagic effusion, now mentioned as localized in the mucous membranes, are to be deprecated as exercising a pernicious influence, seriously aggravating ordinary cases and fatally jeopardizing the issue of severe ones.

Effusive and inflammatory complications are also encountered in the serous structures, and usually in cases of great severity, though they occasionally present themselves when the more common localized phenomena of scurvy are not particularly prominent. These complications may be marked by a gradual accession, or they may rapidly arise and involve the patient, just before in apparent security, in the greatest peril. These incursions are almost always attended by febrile exacerbations and the usual grouping of clinical characters denotive of the same pathological conditions arising under ordinary circumstances. The local complications may either affect the pleura or pericardium, or both. In Karairajew's[18] 60 autopsic examinations pericardial effusions were noticed in 30, pleural in 30, pericardial and pleural in 6, peritoneal in 7, and arachnoidal in only 1. The exudations are sero-sanguinolent or fibrinous in character, and sometimes reach the inordinate quantity of four or five pounds, occasioning the patient the utmost distress and embarrassing the respiratory and circulatory functions. Although these augment in a high degree the risk to life, yet under prompt and appropriate treatment recovery may take place and the effusions vanish with surprising rapidity.

[Footnote 18: Himmelstiern, Beobachtungen über den Scorbut, S. 50, Berlin, 1843.]

Hemorrhagic extravasation into the nervous centres is a very rare occurrence. It has not been as yet recorded as having occurred in the brain-substance itself, but has in several instances been noted between the meninges, producing headache, dizziness, vertigo, and sometimes somnolence, delirium, and coma. Opitz[19] relates an interesting case in which convulsions suddenly occurred with unconsciousness, followed by hemiplegia of the left side of the body and the corresponding side of the face. After twenty-four hours consciousness returned and the paralysis disappeared. There were, however, headache and hyperæsthesia of the upper extremities present; twelve days later these also receded, and the patient finally recovered. The same author records paralysis as occurring in one case from extravasation into the spinal meninges. Samson observed an instance in which a fibrinous effusion formed upon the sciatic nerve, with consequent pain.

[Footnote 19: Prag. Vierteljahrschrift, S. 153, 1861.]

{181} In the circulatory system symptoms always of threatening and often of fatal import may arise: embolism may occur at various points, particularly in the lungs and spleen, occasioning hemorrhagic infarctions, which have undoubtedly been the occasion of the sudden deaths sometimes observed in scorbutic cases not apparently of a very dangerous form nor attended with an excessive degree of exhaustion.

The urinary system supplies no prominent symptoms; the statements as to the condition of the kidneys and the composition of the urine are contradictory. The urine not infrequently contains albumen, particularly in severe cases, but this is by no means indicative of corresponding changes in the renal structure: on the contrary, this may be found after death to be apparently free from disease.

The conclusions that would seem to be authorized by the statements of various authorities are that the quantity of urine passed is decreased, as well as that of the urea, while the amounts of the albuminoid and mineral matters are increased.

Physical examination will reveal the frequent occurrence of enlargement of the spleen, independent of malarial influences, and Krebel has encountered one case in which the liver was involved in inflammation.

Some derangement of the visual organs is present in many cases. Foltz, in the epidemic on the Raritan, reported four cases of nyctalopia and two of hemeralopia, and other affections of the eye, such as conjunctivitis, induration and irritation of the ciliary margins of the lids, with a copious and acrimonious discharge, these conditions being obviously due to the scorbutic diathesis. Medical Director J. Y. Taylor, U.S. Navy, in a private communication to me states that hemeralopia was a frequent premonitory symptom of scurvy that occurred in the U.S. sloop-of-war Decatur in 1854 during a laborious and tedious passage of three months through the Straits of Magellan. The men were overworked and much exposed to cold and wet, and part of the time were on diminished rations. The hemeralopia was at first erroneously attributed to the reflection from the snow and glaciers--a species of snow-blindness--but other phenomena speedily appeared in a majority of the causes: a subacute inflammation, with considerable pain and swelling of the small joints, especially those of the toes; sore and tender gums, although only a few progressed so far as to exhibit sponginess or bleeding; and debility, depression, anxiety, and insomnia. In a few cases the blindness was so complete as to render their subjects almost helpless after sunset. This was the most pronounced and remarkable symptom and the one most complained of. These incipient scorbutic symptoms were promptly arrested by the free use of wild celery (Apium graveolens), which was found growing abundantly in sheltered places. The short rations were also supplemented advantageously by mussels (Mytilus edulis) whenever they could be obtained. A few weeks later the crew appeared to be in ordinary health.

Hemorrhage may occur under the conjunctiva, raising it into small pouches; into the anterior chamber, causing iritis and adhesions; and, finally, into the choroid and vitreous humor, exciting a general inflammation of the entire organ.

Dulness of hearing and buzzing in the ears have also been signalized as occasional symptoms of scurvy.

{182} The phenomena of fever are always absent during the course of uncomplicated scurvy, the temperature of the mouth sometimes falling as low as 92° F., and being always one or two degrees lower than normal. It is only in the later periods of the disease, when pathological processes most often supervene in the internal organs, that an elevated temperature and the other ordinary symptoms of fever are manifested. The lowered vital resistance of scorbutic subjects particularly disposes them to the incursions of fevers, especially those of malarial and typhoid types: hence in the low, marshy districts of Northern Europe and in sections of country afflicted by famine and overcrowded dwellings these complications are very common.

DIAGNOSIS.--Little or no difficulty will be encountered in discriminating scurvy from other diseases under the circumstances that usually surround its development and prevalence. These circumstances are altogether peculiar and characteristic, and involve the absence of succulent vegetable food as the prime factor, and exposure to cold, fatigue, mental despondency, or other depressing influences as accessory in its production. This combination of causes has been usually witnessed in all the outbreaks of scurvy in camps, besieged towns, on shipboard, particularly on ships in Arctic service.

Sporadic cases may escape immediate identification in the absence of some of these circumstances, but a close attention to the symptoms will surely lead to a correct conclusion. The scorbutic cachexia denoted by the sallow or earthy hue of the skin; the spongy gums; the discoloration of the surface; pains in the limbs and joints; the sense of weariness, and, later, the exhaustion, dyspnoea on the slightest exertion; the bloody and fibrinous effusions into the connective tissues and muscles about the joints, and into the pleuræ, pericardium, and peritoneum; the stiffness and contraction of the legs,--furnish a complexus of phenomena not met with in any other disease than scurvy. The discoloration of the skin in purpura, leucocythæmia, anæmia, chlorosis, and hæmatophilia, or other conditions involving hemorrhagic extravasation, are easily discriminated from those of scurvy when taken in connection with the other symptoms and the history of those diseases. In the beginning of scurvy the pains in the back and limbs might divert the attention to rheumatism, but an examination at this early stage will, in all likelihood, disclose the peculiar gingival and cutaneous lesions of scurvy.

The rapid improvement of scorbutic cases under a fruit and vegetable diet is also a noticeable feature not witnessed in any of the foregoing diseases.

PROGNOSIS.--The prognosis of scurvy is always favorable in the early stages, and even in the very worst recovery occurs under improved hygienic surroundings with remarkable promptness and certainty. It must not be overlooked, however, that sudden death may occur in seemingly light cases from failure of the heart's action or from embolism. There is a ready disposition to the recurrence of the disease under slight causes, and it may so impair the health as to lead to the development of other fatal maladies. The gravity of the case is to be gauged not so much by its seeming severity as by the accessibility of proper food-supplies, for without these the worst results may be expected. Where the case is embarrassed with complications of the respiratory and circulatory {183} organs, involvement of the bones, and intercurrent diseases, the outlook becomes correspondingly grave.

Throughout the world, in recent times, greater areas of territory are devoted to agriculture and horticulture, and the products are distributed over wide extents of country by the increased facilities of communication by the highways and railroads, so that it would now be impossible for an epidemic of scurvy to devastate a region of country so provided as it did a century ago, or might do and has done in regions of country where tillage is neglected and communications are cut off by an absence of roads from more productive centres, as in Southern and Eastern Russia.

Hygienic improvements that have almost stamped out scurvy on shore have also done good service for mariners, and thousands of ships now cross the ocean on long cruises with perfect security from the disease. In the naval services of the world, as has been already shown, the disease is rarely encountered, and it is greatly diminished in the merchant marine, from which, it is hoped, in a few years, by a more rigid enforcement of existing laws for the protection of sailors, it may also entirely disappear. Even in exceptionally long and arduous cruises, as in the Arctic regions, the disease may be arrested, as was the case with the Jeannette, which was drifted about, locked up in ice, for sixteen months, yet only a single case of scurvy appeared.

It is of the first importance to enlist a healthy crew for long voyages, free from previous syphilitic, scorbutic, or other constitutional taint; then, by observing proper hygienic precautions, to maintain their health. One of the prime factors in securing this result is a suitable dietary. The improved methods of preserving food afford facilities for storing up adequate quantities of both kinds, animal and vegetable, to last the cruise. To economize these stores it will be well to start with a stock of live animals and recent vegetables, such as can be now had in almost any quantity in any considerable maritime city, and not until these are consumed are the canned and preserved supplies to be opened. All the ordinary meats, as beef, mutton, veal, and lamb; most vegetable products, as asparagus, beans, peas, potatoes, and a great variety of fruits, as peaches, plums, berries, etc., are obtainable at moderate expense, and should form an integral portion of the ration. Eggs can be easily preserved so as to keep for months by simply packing them in plaster or in salt, and they furnish a valuable and acceptable article of diet. Among articles of great nutritive value milk takes high rank, and it can be preserved sweet and pure indefinitely. Sauer-kraut is an antiscorbutic of considerable virtue, and should not be overlooked in laying in stores for a distant cruise. Cheese and oatmeal will be found useful additions to the ordinary ration.

It may be proper to state in the event of the occurrence of scurvy and the exhaustion of the fresh vegetable stores that various quickly-growing vegetables, such as mustard, radishes, turnips, and cresses, could be cultivated on shipboard if seeds are provided.

With such a varied dietary, comprehended in the above enumeration, it would be impossible for scurvy to invade the ship's company, especially when aided by other wholesome agencies, as cleanliness, well-ventilated and dry sleeping rooms, and clothing adapted to the weather. The antiscorbutic virtues of lime-juice were known long ago, being mentioned by {184} Albertus in 1593, but it was not until many years later that it became an integral part of the English navy-ration. The law requires it to be carried on board all merchant vessels, and to be served out ten days after the crew has been living on salt rations. The juice keeps well if properly prepared and preserved from contact with air, especially when fortified with a small quantity of alcohol, the usual strength being about 10 per cent. It should be carried in vessels containing just enough to furnish a few days' rations to the whole crew, by which plan only a small amount need be exposed to the decomposing influence of the air. The juice can be reduced by evaporation to a very small bulk. This method was adopted in supplying the Arctic cruiser Rodgers.[20] The juice was reduced to a paste, each pound of which represented one gallon of the solution of the ordinary strength. It has also been used in the form of lozenges and biscuit. It may be stated that great reliance has been placed upon malt, the acid wines, and cider as good antiscorbutics.

[Footnote 20: Report of the Surgeon-General of the Navy for 1880.]

In connection with the food-supplies it is proper to mention those influences of a depressing character which have a tendency to favor the development of scurvy. The first is dampness in the sleeping apartments of the men. This should be prevented by ventilation, drying stoves, and taking care that no wet garments are permitted to remain in the apartments. They should be taken off immediately and hung outside to dry, and under no circumstances should the men be permitted to sleep in them, as is sometimes done.

Exposure to cold is unavoidable under certain conditions, and the men should then be protected by proper clothing adapted to the weather. Protracted fatigue is a third favoring circumstance, and the crew should be spared all the strain of hard work possible, especially in high latitudes. The apartments should also be kept well ventilated and scrupulously clean; and, lastly, depressing mental emotions, which are so apt to arise from exposure to danger and want, should be dispelled by cheering assurances, constant occupation, and whatever amusements can be had. These are the chief influences which are to be considered in adopting measures to prevent the occurrence of scurvy in communities, armies, on shipboard, or in persons confined in houses of detention.

The therapeusis of scurvy presents no intricate problems for solution. Its origin in dietetic errors is admitted by almost common consent, and it is surprising with what rapidity patients apparently beyond hope of recovery gather health and strength with a change in the character of the food. This is indispensable in the treatment, as drugs have little or no curative influence without it; and, therefore, the first object should be to supply the patient with lemon-juice or acescent fruits and fresh vegetables, as garlic, mustard, cresses, sorrel, nasturtium, taraxacum among the wild plants, and potatoes, onions, turnips, beets, radishes, etc. among the domesticated plants. And in conjunction with these fresh meats, in the form of soups if the solids cannot be masticated, may be used with advantage. Ordinarily, the dietetic treatment alone will suffice to re-establish the health. Should, however, convalescence be delayed, the vegetable bitters with the mineral acids and ferruginous tonics and quinia will furnish useful adjuvants. These are the standard remedies; others have been recommended at various times, as the juice {185} of the maguey, a Mexican plant, potassium nitrate alone or combined with vinegar, tincture of cantharides, etc.

Attention will often be required to the various scorbutic complications, especially stomatitis, which is always a source of discomfort and suffering. One of the best local applications for this is pencilling the parts with a solution of nitrate of silver, which often affords marked relief. Mouth-washes, composed of solutions of chlorinated lime, potassium permanganate, carbolic acid, are beneficial by suppressing foul odors, exercising local stimulative action upon the gums, and promoting healing. Should ulceration attack the legs, as is often the case, the application of mild astringents and stimulative ointments will be all that is required. The parts should, of course, be kept clean and protected from irritation by protective dressings.

Hemorrhages from the nose, gums, stomach, bowels, or into the serous cavities should be treated upon the general principles applicable to their character, as the local use of cold, astringents, and the internal administration of hæmostatic agents--lead acetate, ergot, tincture of iron, and other remedies, vegetable and mineral, of this class. In desperate cases effusions into the chest, threatening death by interfering with the respiratory and circulatory organs, may render operation necessary as the last resort for their removal.

During the treatment it is important to obviate any sudden or severe strain upon the heart by premature movements or exercises, as this is fraught with danger.

{186}

PURPURA.

BY I. E. ATKINSON, M.D.

It has been customary with authors to describe under the general heading Purpura a number of affections presenting as a common symptom the extravasation of blood into the tissues, more especially of the skin and mucous membranes, quite irrespective of etiological or pathological considerations. Thus, the tiny ecchymoses caused by the bites of fleas have been denominated purpura pulicosa; the larger bruises resulting from external violence, purpura traumatica; the extravasations occurring in the course of scurvy, purpura scorbutica; those encountered in malignant small-pox, purpura variolosa; and so on. These affections, differing widely in nature, possess as a common symptom the escape of blood from the vessels into the tissues. It is evident, therefore, that in the sense often employed the term purpura is used to describe a symptom or symptoms common to a variety of non-related maladies.

If there be a peculiar morbid process having for its constant and characteristic symptom the spontaneous escape of the blood from the blood-vessels, it is plain that interstitial hemorrhage from external violence or from the action of a definite poison circulating in the blood and disorganizing it and its containing vessels, as in phosphorus-poisoning, or from the influence of certain zymotic diseases, should not be designated by the title properly belonging to a substantive malady. The question, therefore, is: Are there groups of symptoms indicating morbid action of definite character, but of varying intensity, to which the name purpura may with propriety be applied?

In the present light of pathological science it is impossible to answer this question in the affirmative without considerable qualification. It must be confessed that we do not possess a knowledge of any definite chain of morbid processes constituting a distinct disease that may be designated as purpura. And yet we are able to recognize a set of symptoms varying greatly in intensity, from the most trivial petechial eruption to profuse and fatal hemorrhages, accompanied by a train of manifestations which we are unable to connect with any of the causes already spoken of, and which, indeed, depend upon no fixed exciting cause with which we are acquainted. It may be eventually proven that purpura, even as we understand it, is merely a set of phenomena due to widely-differing influences acting upon the blood and blood-vessels, and that the term will disappear from our nomenclature as indicating a disease, but will be preserved as denoting a symptom. For the present, {187} purpura is understood to be a group of symptoms characterized by the effusion of blood into the tissues of the body, or upon its free surfaces, or into its serous cavities, which seem to arise spontaneously, and for which we are unable to assign a definite cause. With this view of the nature of purpura it becomes necessary to exclude from present consideration blood-extravasations from internal or external violence, the action of the specific principles of contagious or infectious fevers, the dyscrasia of scurvy, the influence of poisonous substances, and, in a word, any of those affections of which the escape of blood from the vessels constitutes an epi-phenomenon.

Purpura may be conveniently considered as presenting three varieties: 1, purpura simplex; 2, purpura hæmorrhagica; 3, purpura rheumatica.

These three forms of the disease are not distinguished by sharply-outlined differences, but merge the one into the other, now one, now another set of symptoms predominating. To these may be added, likewise for convenience, three sub-varieties--purpura urticans, purpura papulosa, and purpura nervosa. The difference between these forms of purpura should not be considered as of more than clinical import. Whatever variations present themselves may with probable propriety be ascribed to complicating influences.

PURPURA SIMPLEX.--This is the mildest form of purpura, and may in many cases readily escape observation. It may begin abruptly, in the midst of health, without the slightest subjective symptom, or the extravasations may be preceded for several days by some discomfort, aching of limbs, sluggishness, anorexia, even a small amount of fever. The eruption usually appears first upon the lower extremities, preferably the flexor surfaces of the thighs (Duhring), but frequently upon the legs. It extends from these points to the upper extremities and trunk, usually sparing the face. The lesions vary in size from that of a pin-head to that of a fingernail (petechiæ), or they may be linear (vibices). They remain discrete, and do not increase in size throughout their course. Each spot of hemorrhage will endure for from one to two weeks. At first the lesions are of a livid red color, and declare their extra-vascular nature by remaining unaltered when subjected to pressure. The color of these spots changes, as in ordinary ecchymosis, in consequence of the metamorphoses of the hæmatin preparatory to its final absorption, from crimson to purple, to blue, to green, to yellow, and finally fades away. When recent, the spots appear sharply outlined, with sometimes a faint encircling zone of hyperæmia, but as they become older their margins grow indistinct. While the early lesions slowly disappear, others continue to develop, and the affection may thus be protracted for weeks. At times the petechiæ appear in crops, recurring every few days, the patient at one time apparently nearly well, at another time worse than ever. Finally, the symptoms definitely disappear, to return no more, or they pass into those of other forms of purpura. During the course of purpura simplex the blood-vessels of the skin alone are affected, the deeper tissues and mucous membranes probably remaining unchanged.

Throughout the attack the general health may--usually does--remain good. As an occasional symptom there will be observed a few vesicles or blebs, containing blood, upon the skin. The extent of the general eruption may vary from a few scattered petechiæ to a copious and startling {188} number of purpuric spots. The maintenance of the upright position tends to perpetuate the evolution of the lesions.

In elderly persons purpura simplex is sometimes observed, and has been described by many writers as purpura senilis. Hillier, following Bateman, describes it as occurring in old women "upon the outside of the forearms in successive dark, purple blotches of an irregular form and various magnitude."[1] Aged men as well as women are liable to the affection, which may quite as well appear upon the lower extremities of either sex. It is altogether likely, however, that in such cases degenerations of the vascular walls alone may cause the extravasations.

[Footnote 1: Reynolds's System of Medicine, vol. i. p. 792.]

PURPURA HÆMORRHAGICA (MORBUS MACULOSUS WERLHOFII).--In this form of purpura there are added to the symptoms of purpura simplex hemorrhages into and from the various mucous tracts, the nasal, faucial, pharyngeal, gastric, intestinal, renal, uterine, rarely the pulmonary mucous membranes, and exceptionally into the various serous membranes and cavities. It may begin abruptly, in the midst of apparently vigorous health, or after premonitory symptoms extending over several days, vague sensations of discomfort--headache, pains, anorexia, indisposition to exertion, and the like--or it may occur as a transition from other forms of purpura. Usually there is no fever.

The hemorrhagic spots upon the skin appear much as in purpura simplex, though the lesions are larger, acquiring the size of coins or even of the palm of the hand. Spots soon appear upon the visible mucous membranes, and free hemorrhages occur; indeed, the latter may be the first symptom observed. Epistaxis is of most common occurrence, but bleeding from the mouth, stomach, and intestines almost as frequently results. The gums are almost constantly affected, and upon inspection these may be found covered with blackish scabs, upon removal of which the mucous membrane will be found pale and not swollen--an important point in diagnosticating this affection from scurvy. Vesicles and blebs filled with blood form both on the skin and mucous membranes. They quickly rupture and discharge their contents. Bleeding from the stomach and intestines is revealed--in the former case by the vomiting of a brownish material resembling coffee-grounds; in the latter case by the passage of black, tar-like evacuations. Pulmonary hemorrhage is to be distinguished from hæmatemesis by the frothy and arterial character of the blood. Hæmaturia may proceed from any part of the urinary tract. Bleeding from several parts may occur at the same time, and may be very copious.

In the mucous membranes extravasations of greater or less extent may occur, as in the derma. Into the serous membranes they may take place with or without effusion into serous cavities. It is only, however, in cases that will almost certainly end fatally that the effusions into these cavities are encountered. Hemorrhages into the substance of the lungs, into the brain and other viscera, as well as into the tissues generally, are occasionally observed.

At the outset of these bleedings the general health of the patient may appear unimpaired, and if they be few in number and moderate in extent but slight evidences of debility may be shown throughout the attack; but it is often the case that the loss of blood is excessive and long continued, and symptoms of profound anæmia supervene. The {189} patient becomes greatly exhausted; intense pallor is developed, shortly followed by general oedema. Attacks of syncope appear, and in fatal cases--which are not common--death results from asthenia. This result may occur after a few days from the profuseness of the hemorrhage; usually, however, only after several weeks. Throughout the attack the cutaneous lesions continue to develop, either irregularly or in successive outbreaks, scattered over the general surface, involving the face less frequently than other parts. These spots undergo the color-changes peculiar to extravasated blood, and may be seen in all the stages of involution in the same patient. Fever, usually absent throughout the attack, may appear at the height of the affection, but does not run high. Local inflammations are exceedingly rare. In favorable cases recovery follows the gradual mitigation and disappearance of the symptoms, but relapses frequently occur, and convalescence may be retarded for months.

PURPURA RHEUMATICA (PELIOSIS RHEUMATICA).--Schoenlein in 1829 described as peliosis rheumatica an affection in which the symptoms of purpura simplex were associated with pain and often with effusion into the joints, especially those of the knee and ankle. He considered it as an independent malady. This opinion has been shared by Fuchs, Hebra, Kaposi, Neumann, and many others. Kaposi[2] regards it as related to erythema nodosum, with which affection, indeed, it possesses some features in common. It probably, however, constitutes a complication of ordinary purpura. That it is not primarily rheumatic is shown by the almost invariable absence of many of the symptoms characteristic of rheumatism; that it cannot be an independent affection appears from its intimate relations with other forms of purpura.

[Footnote 2: Hautkrankheiten, 1880, p. 277.]

Purpura rheumatica commonly begins with malaise, anorexia, debility, sometimes with mild fever. The patient is soon attacked with pains, of a more or less acute character, in the joints, especially the knees and ankles. There may be some effusion into the joint and cutaneous oedema. After a few days the nature of the complaint will be revealed by an eruption of petechiæ, first near the painful joints, but soon extending, involving in many cases even the head and trunk. The eruption may be at first slightly elevated and surrounded by a fine halo of hyperæmic injection.

The pains usually subside upon the appearance of the eruption, and the malady may be completed after a single outbreak. More commonly new joint-pains are experienced, fresh crops of petechiæ appear, and the trouble may be prolonged for weeks, even months, the patient meanwhile suffering not very greatly in general health. The lesions may be cutaneous only; rarely bleeding from mucous surfaces will occur (Scheby-Buch). Albuminuria may be present (Kaposi). An annual type is said by Kaposi, Neumann, and others to be sometimes observed, the spring and autumn being the usual seasons for the outbreaks. This is supposed to indicate a relationship with erythema nodosum and multiforme. Cardiac murmurs have been detected in the course of purpura rheumatica,[3] but these were probably anæmic or antedated the purpuric symptoms. Purpura rheumatica never seems to result in endo- or pericarditis.

[Footnote 3: Kinnicutt, Archives of Dermatology, i. p. 193; Mollière, Ann. de Dermatol., v. p. 44.]

SUB-VARIETIES.--Henoch[4] and Couty[5] have described a form of {190} purpura mostly observed in children, in whom rheumatoid pains occur along with colic and vomiting of greenish or bilious matter, tenesmus, and sometimes with loss of blood from the bowels. The disease may be protracted throughout months by relapses. Cutaneous oedema frequently occurs. Couty regards it as a form whose peculiarities justify its assignment to a position of its own. The cause of the associated train of symptoms is supposed (Couty) to reside in the sympathetic system, and the name purpura nervosa is proposed for it. So many features of ordinary purpura are manifested in these cases that it seems better to consider them as examples of ordinary purpura complicated with gastro-intestinal derangement. It has been suggested that the nausea, vomiting, and abdominal pains may result from extravasation of blood into the peritoneal tissue.[6]

[Footnote 4: Berl. Klin. Wochenschr., 51, 1874.]

[Footnote 5: Gaz. Hebd., 36 et seq., 1876.]

[Footnote 6: Immermann, Ziemssen's Cyclopæd., vol. xvii. p. 265.]

In the course of purpura there is frequently observed, more especially in purpura simplex, a wheal-like arrangement of the eruption--such, indeed, as occurs in urticaria. The term purpura urticans has been given to this sub-variety, which may or may not be accompanied by itching. Scheby-Buch has suggested that the urticaria may, with more propriety, be attributed to the gastric disturbances that so often accompany the forms of purpura presenting it.[7] The wheals are usually seen upon the lower extremities, but may appear elsewhere. A considerable degree of oedema may be present, particularly in lax tissue, such as that of the scrotum, eyelids, etc.

[Footnote 7: Deutsche Arch. f. Klin. Med., B. xiv. p. 490.]

Purpura papulosa (lichen lividus, Willan) is a form of purpura where, in the midst of ecchymoses, livid papules appear. These probably depend upon a large amount of hemorrhage occurring within a limited space, most often surrounding the orifices of hair-follicles, because these are supplied with a capillary network that comes directly from the deeper layer.[8] They are formed most abundantly on the legs of scrofulous, cachectic persons who have purpura. Care must be taken to distinguish this form of purpura from erythema multiforme and erythema nodosum, where blood is usually extravasated secondarily into the tissues. Those cases only where the purpura is primary should be recognized as purpura papulosa.

[Footnote 8: Hebra, Skin Diseases, New Syd. Soc. Transact., ii. p. 425.]

The purpuric effusion appears to act as an irritant upon the tissues, and to excite inflammation. Gangrene of the mucous coat of the intestines has resulted from extensive hemorrhagic extravasations, and from a similar cause cutaneous gangrene has been known. These complications, however, are rare.

ETIOLOGY.--The immediate causes of purpura are quite unknown. Both sexes and persons of every age are affected by it. While it is most often seen in debilitated subjects, those in vigorous health possess no immunity. It has often been observed during convalescence from other maladies. It cannot be said that those who are miserably clothed, fed, and lodged are especially predisposed to attacks of purpura. Between purpura and hæmophilia, etiologically, there are many points of difference. Purpura is not hereditary, nor is there a purpuric diathesis in the strict sense of the term. Some persons, indeed, seem to possess a {191} predisposition to the disease, and some authors claim for purpura rheumatica a distinct annual type. This, however, is not at all certain.

Recently it has been claimed that purpura hæmorrhagica depends upon the presence of a minute organism in the blood. Petrone[9] injected blood drawn from patients with this disease under the skin of rabbits, producing widely-distributed hemorrhages. In the blood of these individuals and of the injected rabbits micrococci and bacilli were detected. Watson Cheyne[10] also describes a plugging of the capillaries with bacilli. These were 1/7700 of an inch in length and 1/20000 of an inch in diameter, and were arranged in colonies. In another case there were found micrococci arranged in chains. These swarmed in the capillaries and some larger vessels, and sometimes completely blocked them. Although an origin in infection has thus been claimed for purpura hæmorrhagica, the fact that more than one variety of micro-organism was observed cannot fail to excite suspicion of, possibly, erroneous observation.

[Footnote 9: Lo Sperimentale, 51, 1883.]

[Footnote 10: Lancet, i., 1884, 344.]

PATHOLOGY.--In the foregoing description those extravasations of blood due to simple mechanical violence, as from flea-bite, and sudden increase of blood-pressure, as in the effort of coughing in whooping cough, also from the deleterious influence exerted upon the blood-vessels and blood by certain drugs, the specific fevers, Bright's disease, and the like, have been excluded. Only those have been considered where the effusion of blood seemed to occur spontaneously, and the symptoms to result from some peculiar but not understood morbid process. The hemorrhage is but a symptom; the process by which it is brought about depends upon some change in the blood or blood-vessels. We do not know what these subtle changes are. The blood of purpuric patients has been carefully examined, but, with the exception above mentioned, no definite changes have been discovered. Immermann[11] found during the first stage of the disease the blood-corpuscles perfectly normal in appearance, the white corpuscles subsequently slightly exceeding the red in number--a simple result of copious hemorrhage. No stated chemical changes in the blood are known in purpura, nor is it known how the blood escapes from the vessels. It undoubtedly escapes through alterations in the vascular wall, but it is also true that red blood-corpuscles, as well as the pale ones, may find their way in considerable numbers through the unruptured wall of the vessels, per diapedesin, as was first suggested by Velpeau, but definitely determined by Stricker. The causes of this migration are obscure. Immermann[12] asserts that a fatty degeneration of the vascular tissues and of the muscles takes place. This, however, is manifestly a result of the loss of blood, and not its cause. Dr. Wilson Fox[13] found extensive albuminoid disease of the muscles and capillaries of the skin; but the albuminoid degeneration involved several organs of a patient with syphilis, and the purpura was certainly secondary to the morbid conditions. Rigal and Cornil[14] think that the hemorrhages are a result either of sympathetic irritation or of diminished action of the vaso-motor centre. It is indeed altogether likely that the cause will ultimately be found to reside in the vaso-motor system.

[Footnote 11: Ziemssen's Cyclop., xvii. p. 258.]

[Footnote 12: Loc. cit.]

[Footnote 13: Brit. and Foreign Med.-Chir. Review, Oct., 1865.]

[Footnote 14: L'Union Méd., 5, 6, 7, 1880.]

{192} DIAGNOSIS.--The affection bearing the closest resemblance to spontaneous purpura is scurvy; indeed, its supposed relationship to this disease has given purpura one of its synonyms, land scurvy. The two affections, however, are probably without the slightest relationship. They possess in common the hemorrhagic symptoms, both in the tissues and from free surfaces, but the resemblance does not extend much beyond this. Scurvy depends upon deprivation of fresh vegetable food and the use of unsuitable and insufficient food generally, and upon bad hygienic surroundings. Purpura may--frequently does--appear in broken-down constitutions, but it equally attacks the strong and vigorous, while the character of food exerts no special influence on its production. Scurvy only follows long-continued privations and as a culmination of a train of distressing symptoms. Purpura appears in the midst of health, or after brief premonition, or during convalescence from totally unrelated diseases. In scurvy there is a decided tendency toward ulceration, which is absent in purpura. In scurvy the mouth and gums inflame and ulcerate, the latter becoming swollen, spongy, and of a bluish-red color. In purpura, ulceration of the buccal mucous membrane does not occur, and the gums are pale and intact. The curative influence of fresh vegetables, lime-juice, etc. in the treatment of scurvy is not observed in purpura. It has been claimed that purpura is but a mild degree of scurvy: this cannot be so, for we may have a mild scurvy or a severe, even fatal, purpura.

The hemorrhagic diathesis, or hæmophilia, presents points of analogy with purpura. Here, however, is found the almost constant history of heredity and the implication only of persons of the male sex. The disposition to bleed at all times upon the receipt of the smallest injury is quite unlike the suddenly-developed and transitory hemorrhages of purpura, which are also more generally distributed.

With the secondary hemorrhagic effusions and ecchymoses that occur in conditions of profound alterations of the blood and blood-vessels in cases of malignant small-pox, scarlatina, typhus fever, etc., and in some cases of poisoning, as from phosphorus, spontaneous purpura presents identities, but the history of the complaint and the condition of the patient will prevent error. A knowledge of the circumstances will serve to distinguish purpura simplex from the petechiæ and small ecchymoses produced by fleas, by diminished atmospheric pressure, by coughing, in the course of Bright's disease, etc.

Purpura rheumatica presents, as has been shown, many points of resemblance to erythema multiforme and erythema nodosum. The mild fever, the joint-pains, the extravasations of the latter affections, are much like the symptoms of this form of purpura. The nodular, inflamed, tender condition of the lesions, their location--frequently upon the extensor surfaces of the extremities--their course and duration, usually serve to identify erythema nodosum, while with erythema multiforme it is usually not difficult to observe its essentially inflammatory character. Scheby-Buch has shown the difficulties often opposed to the differentiation of purpuric lesions and ecchymoses due to violence.[15] Where the petechial eruption of purpura simplex is well marked, where the internal hemorrhages of purpura hæmorrhagica are copious, the inquiries of the observer will usually lead him to correct conclusions. Where the {193} ecchymoses are larger and upon exposed parts of the body, the diagnosis from the lesions alone becomes impossible, and due consideration of all concomitant circumstances is essential. It should be remembered that in purpura very slight violence may call forth extensive ecchymosis. This circumstance has important medico-legal bearings.

[Footnote 15: Viertelj. f. Dermatol. und Syph., 1879, p. 99.]

PROGNOSIS.--Purpura usually terminates favorably. Its course runs from two to six weeks, rarely longer. Relapses and remissions are frequent. Purpura simplex is of very little gravity, and need excite little apprehension. Purpura rheumatica almost always ends in recovery; fatal terminations, however, have been known. Purpura hæmorrhagica is of much more serious import. Even here, however, though the patient may fall into profound debility from loss of blood, recovery is the rule, the symptoms gradually diminishing in severity until health becomes re-established. In fatal cases death ensues after prolonged and profuse losses of blood. Purpura may subside after a single outbreak or many relapses, and recrudescences may occur extending through months. Anæmia may persist long after the disappearance of purpuric symptoms. A tendency to purpura may be shown at irregular intervals for years, and even throughout life.

TREATMENT.--Very mild cases of purpura simplex require no treatment, not even confinement within doors. The patient is often first made aware of his disease by accident; doubtless it frequently escapes detection altogether. It has been observed that purpura often appears upon the lower limbs of convalescents from other diseases when they first essay the upright position. Relapses of purpura also frequently appear as the patient leaves his bed. We have here an important indication for treatment--viz. the maintenance of the recumbent posture in cases of any degree of severity. Fresh vegetables and vegetable acids do not have the same happy influence as in scurvy. It is manifestly important that appropriate food should be administered in sufficient quantity, both to improve the general health and to repair the exhausting losses of blood. Milk is an exceedingly valuable article of diet in these cases, being but little apt to irritate the mucous membrane of the alimentary canal.

The patient should be guarded against violence. Injuries that may be of no consequence to healthy persons may excite in the purpuric profuse hemorrhage, free or interstitial. Violent emotions and physical efforts should be avoided, as in stimulating the heart's action a condition of increased blood-pressure ensues that may readily result in extravasation.

There are no remedies that exert a specific influence over purpura, and yet quite a number have enjoyed, and still enjoy, high reputation in controlling the symptoms. Probably the most frequently employed remedy against purpura is sulphuric acid, preferably the aromatic sulphuric acid, in doses of from 15 to 20 drops, diluted well with water and administered every third or fourth hour. It is certainly an agent of value, though some authors maintain that it has no efficacy (Immermann). Acetate of lead undoubtedly exercises an influence over the course of the disease. More recently, ergot has been employed. Its use has been highly extolled by Buckley and others. Very large doses may be given. The hypodermic use of ergotin has been followed by results most gratifying to those employing it. Oil of turpentine has enjoyed considerable reputation. A remedy that undoubtedly has a good effect is iron, both as {194} exercising a controlling action over the bleeding and as assisting to repair the resulting anæmia. The tincture of the chloride is the most suitable preparation, and may be given in large doses (from minim xx to fluidrachm ss), well diluted, every fourth hour. Care must be exercised to avoid irritating the digestive organs with it. Formerly, venesection was employed to prevent the occurrence of hemorrhage, but its efficacy in this direction is at least doubtful, and cannot but help to intensify the disastrous consequences of severe and protracted attacks.

The various complications that may arise, as well as the general results of purpura, must be treated symptomatically. For the mucous membranes astringent washes should be used, and in favorable situations the tampon may sometimes be employed with profit. In purpura rheumatica the arthritic pains will be alleviated by anodyne liniments and plasters, and the often accompanying abdominal pains and colic by anodynes internally administered. Hæmatemesis, hæmaturia, etc. must be treated upon general principles. The results of profuse hemorrhage must be combated with stimulants. Transfusion of blood has been proposed and practised for the extreme anæmia that sometimes occurs, but without encouraging results. If necessary, the bowels may be kept free by mild aperients. In severe cases rest in bed should be rigidly enforced until after the establishment of convalescence. Quinia, iron, and nux vomica are indicated above all other remedies for the anæmia resulting from an attack of purpura.

{195}

DIABETES MELLITUS.

BY JAMES TYSON, A.M., M.D.

Diabetes mellitus is a term applied to a group of symptoms more or less complex, of which the most conspicuous is an increased flow of saccharine urine--whence the symptomatic title. It is associated with a derangement of the sugar-assimilating office of the liver, as the result of which an abnormally large quantity of glucose is passed into the hepatic vein and thence into the systemic blood, from which it is secreted by the kidneys. The condition is sometimes associated with alterations in the nervous system, at others with changes in the liver or pancreas, while at others, still, it is impossible to discover any structural alterations accompanying it.

PATHOLOGY AND PATHOGENESIS.--Notwithstanding that this disease has been recognized for two centuries and a half, that abundant opportunity has been furnished for its post-mortem investigation, and that experimental physiology has contributed much information bearing upon the subject, its pathology is still undetermined. Experiment has, however, rendered it very likely that all cases of essential glycosuria--that is, all cases in which saccharine urine is not the direct result of over-ingestion of sugar or sugar-producing food--are accompanied by a hyperæmia of the liver. This hyperæmia, with its consequent glycosuria, can be induced by puncturing or irritating the so-called diabetic area[1] in the medulla oblongata. This area corresponds with the vaso-motor centre, and with the roots of the pneumogastric or vagus nerve in the floor of the fourth ventricle; whence it was at first inferred that this nerve is the excitor nerve of glycosuria. It was soon ascertained, however, that when the pneumogastric was cut, glycosuria ensued only when the central end was stimulated, while {196} stimulation of the peripheral portion was without effect. Whence it became evident that this nerve is not the excitor, but the sensory nerve concerned in glycogenesis.

[Footnote 1: The diabetic area, as marked out by Eckhard, and which corresponds with the vaso-motor area, as defined by Owsjannikow (Ludwig's Arbeiten, 1871, p. 21), is bounded by a line drawn four or five mm. above the nib of the calamus scriptorius, and another about four mm. higher up.]

It was also learned in the course of continued experiment that glycosuria resulted upon transverse section of the medulla oblongata, of the spinal cord above the second dorsal vertebra, of the filaments of the sympathetic accompanying the vertebral artery, upon destruction or extirpation of the superior cervical ganglion, and sometimes, but not always, after division of the sympathetic in the chest (Pavy); also after section or careful extirpation of the last cervical ganglion, section of the two nerve-filaments passing from the lower cervical to the upper thoracic ganglion around the subclavian artery, forming thus the annulus of Vieussens,[2] and after section or removal of the upper thoracic ganglion.

[Footnote 2: Cyon and Aladoff, reprint from the Mélanges biolgiques and Bullétin de l'Académie Impériale de Petersbourg, vol. xiii. p. 91; cited by Dr. Brunton in the Lectures named in note on p. 198; also British Medical Journal, Dec. 23, 1871, p. 731.]

All these operations paralyze the vaso-motor nerves by which, in health, the blood-vessels of the liver are kept in a state of tonic contraction; hence these vessels dilate when the nerves are cut. From the facts named we also learn the path of the glycogenic influence, which must be from the medulla oblongata into the spinal cord, thence by the filaments of the {197} sympathetic which accompany the vertebral artery into the lower cervical ganglion; thence through the annulus of Vieussens into the first dorsal ganglion; and thence through the prevertebral cord of the sympathetic, and branches not precisely determined, to the hepatic blood-vessels as shown by the dotted line in Fig. 3.

I say, by branches of the sympathetic not precisely determined, because our power to produce artificial diabetes fails below the first thoracic ganglion; for section of the sympathetic between the tenth and twelfth ribs, and of the splanchnics, is not followed by glycosuria, although the vaso-motor nerves to the liver are known to pass through them.

According to Eckhard,[3] the phenomena of artificial glycosuria are irritative and not paralytic. This view he believes sustained by his own experiments, according to which if the splanchnics, through which {198} the vaso-motor nerves of the liver pass, are cut prior to the diabetic puncture, not only does this operation fail to produce glycosuria, but it even renders ineffectual the puncture itself as well as the section higher up. But Cyon and Aladoff remind us that it is not mere dilatation of the hepatic vessels, but increased velocity in the movement of the blood, which deranges the sugar-assimilating function and causes glucose to appear in the urine. The vaso-motor nerves of the intestinal blood-vessels also pass through the lower part of the sympathetic and the splanchnics, and section of the latter must cause these blood-vessels to dilate. Now, in rabbits, in which this experiment is usually performed, the digestive canal is very long, and the blood-vessels so capacious that when dilated they hold as much blood as all the rest of the vascular system together, so that when the lower sympathetic and splanchnics are cut, so much blood goes into the intestines that the increased velocity required in the blood-vessels of the liver to produce glycosuria is impossible. But if the vessels of the liver be first dilated by puncturing the floor of the fourth ventricle, section of the sympathetic or of the splanchnics may then be made without arresting the formation of sugar; whence it would appear that the glycogenic influence may still pass through the lower sympathetic and splanchnics.

[Footnote 3: Beiträge zur Anat. und Physiologie, iv., 1859, p. 1; vii., 1873.]

In view of the fact that Eckhard[4] has failed to confirm the results of Cyon and Aladoff, but has traced the glycogenic influence down the spinal cord as far as the fourth dorsal vertebra in rabbits, and even a little lower, and that Schiff[5] has shown that diabetes sometimes results after section of the anterior columns of the cord between the medulla and the fourth cervical vertebra, Dr. Brunton[6] suggests that the vaso-motor nerves of the liver may not always leave the spinal cord to join the sympathetic by the branches accompanying the vertebral artery, but sometimes pass farther down the cord, leaving it by the communicating branches to some of the dorsal ganglia, as indicated in Fig. 4.

[Footnote 4: Beiträge zur Anat. u. Physiologie, viii., 1877, p. 79.]

[Footnote 5: Untersuchungen über Zuckerbildung in der Leber, 1859, S. 108.]

[Footnote 6: Lectures on the Pathology and Treatment of Diabetes Mellitus; reprinted from the British Medical Journal, 1874, p. 12.]

It is evident that an agency involving any part of this tract in such a way as to paralyze the vaso-motor nerves of the liver is capable of producing glycosuria. Such cause may operate upon the central ganglia whence the nerves emanate, as the vicinity of the oblongata and upper parts of the spinal cord or the coeliac ganglion and its branches, including those to the pancreas. Or the irritation may be peripheral and its effects reflex. We have seen that irritation of the central end of the cut vagus will produce glycosuria. Any irritation, therefore, involving the peripheral distribution of this nerve may produce it. Hence embarrassed respiration, whether due to disease of the respiratory passages, strangulation, or inhalation of irrespirable gases and anæsthetics, produces glycosuria in dogs and rabbits; and this symptom has been known to attend these conditions in the human subject. So, too, glycosuria may be produced by such substances as woorara, strychnia, morphia, and phosphoric acid, introduced into the blood and irritating the terminal filaments of the pneumogastrics, or it may be brought about secondarily through the embarrassed respiration these drugs produce. Such peripheral {199} irritation may reside also in the stomach, intestines, liver, or any organ to which the pneumogastric is distributed.

It is not unlikely that irritation of the extremities of sensory nerves other than the pneumogastric may become the cause of reflex glycosuria. Even puncture of the floor of the fourth ventricle itself may be reflex in its operation, the roots of the pneumogastric being thus irritated. The effect of the irritation conveyed to the glycogenic centre is to inhibit the usual tonic influence of the vaso-motor nerve upon the vessel walls. Among the experimental irritations, in addition to puncture of the floor of the fourth ventricle, which produce glycosuria by reflex action, are injuries of the cerebral lobes and cerebellum, optic thalami, cerebral peduncles, pons varolii, middle cerebellar peduncles, and even of the sciatic nerve and brachial plexus; whence it may be inferred that pathological irritation in the same situations may result in a glycosuria, which is temporary or permanent according as the irritation is temporary or permanent.

Finally, there is no reason why an inhibitory reflex action should not originate in the sympathetic itself. When we remember that this nerve is both sensory and motor in function, and that the inhibitory influence to which the heart's action is subject is accomplished through the sympathetic as a sensory nerve and the pneumogastric as a motor, there is no reason why similar results may not be brought about by the sympathetic alone. This being the case, we need not ascribe glycogenic phenomena to irritation in Eckhard's sense--that is, to a direct stimulant action of the irritant upon the vaso-motor nerves of the liver--but may suppose a sensory influence to ascend one set of sympathetic filaments and an inhibitory influence to descend through another.

Dr. Pavy has recently put forward some chemical theories which explain the action of the hyperæmia in producing glycosuria, but they do not account for the hyperæmia itself. In healthy digestion the carbohydrates (starch and sugar) are converted, not into glucose, but into maltose, C_{12}H_{22}O_{11}, dextrin being intermediate in composition. Maltose is absorbed and assimilated, converted into glycogen. So, too, when glucose is ingested as such, it is converted by the glucose ferment into maltose in the stomach and intestines. For the proper production of maltose and its assimilation a good venous blood, producing a maltose-forming ferment, is necessary. In diabetes, in consequence of the dilatation of the arteries of the chylopoëtic viscera, the blood enters the liver too little deoxygenated, and a glucose-forming ferment is produced. The glucose thus formed is not assimilable, but passes off into the circulation and the urine.

MORBID ANATOMY.--Such are some of the facts bearing upon the pathology of diabetes mellitus. Throwing out the milder type of cases, in which glycosuria is the result of an over-ingestion of saccharine and sugar-producing food--and these can scarcely be called instances of essential diabetes--it is evident that glycosuria may be produced in a variety of ways operating through the nervous system; and accordingly we may infer that there is scarcely an organ in close relation with the sympathetic system derangement of which is not capable of producing it. Among these we would naturally expect to find conspicuous alterations in the nervous centres, and yet I have never found changes in these centres after death. At the same time, others have noted meningitis, tubercular {200} and traumatic, apoplectic effusions, and tumors of the brain, especially in the neighborhood of the medulla oblongata. The alterations in the nerve-centres described by Dickinson as the essential morbid anatomy of diabetes I have looked for in vain. These changes are described as a cribriform or porous condition of the white nervous matter, said to be visible to the naked eye. The spaces thus produced are partially occupied by dilated blood-vessels, which, in turn, are surrounded by dilated perivascular sheaths and broken-down nervous matter, into which extravasations of blood have taken place, as evidenced by the presence of pigment-granules. The changes are found in the white matter of the convolutions of the brain, but fewer and larger in the central portions. The corpora striata, optic thalami, pons, medulla, and cerebellum are favorite seats for the largest and most striking holes. In rapidly-fatal cases the cavities are sometimes filled with a translucent, gelatinous substance, containing, besides vascular elements, the globular products of nervous disintegration. In the more chronic forms of the disease, as it occurs in elderly persons, the excavations are usually empty, although the elements of nervous decay are still to be found fringing the margins or collected as an irregular sheath upon the dilated or shrunken artery. There are changes in the cord similar to those in the brain, but less decided. But the most striking alteration in the cord, according to Dickinson, although not always present, is dilatation of the central canal, which in the dorsal and lumbar regions is sometimes expanded to many times its normal diameter, and forms a conspicuous object immediately after the cord is divided.

These alterations have eluded the vigilance of other pathologists who have sought for them in well-determined cases of diabetes mellitus, while they have been found, on the other hand, in the nervous centres when no diabetes was present. In the recent discussion on diabetes at the Pathological Society of London, Douglas Powell[7] seemed to be the only one who was convinced that most of Dickinson's specimens were examples of positive lesions.

[Footnote 7: London Lancet, May 5, 1883, p. 776.]

A hyaloid thickening of the blood-vessels of the brain has been noted by Stephen Mackenzie[8] and Seymour Taylor[9] in some cases, and miliary aneurisms of the retina in one.

[Footnote 8: Discussion on Diabetes, Path. Soc. of London, London Lancet, April 7, 1883, p. 593.]

[Footnote 9: Ibid., Lancet, May 5, 1883, p. 774.]

Of other organs, one of the most frequently found diseased is the pancreas, and, according to Senator, it is fair to assume that disease of the pancreas is present in about one-half of all cases of diabetes. As the result of increased experience, I am inclined to attach much more importance to pancreatic disease as a cause of diabetes than I did a few years ago. Among the changes found is a pseudo-hypertrophy, which consists chiefly in a hyperplasia of the connective tissue, fatty degeneration of the gland-cells, and atrophy of the glandular structure; cancerous disease; calculous concretions in the ducts with or without obstruction; and cystic dilatation.

Facts bearing upon the relation of pancreatic disease to diabetes have been accumulating since Cowley first discovered calculi in the pancreas of a diabetic, and Bright pancreatic cancer in a similar case. Since then {201} instances have multiplied to such extent that it would be unprofitable to enumerate them. But in 1877, Lancereaux[10] communicated to the French Academy of Medicine specimens of profound lesion of the pancreas from cases dying of diabetes mellitus. This, he alleged, constitutes a special and distinctive variety of diabetes, characterized by sudden onset, emaciation, polydipsia, polyphagia, and peculiar alvine dejections. More recently, Depierre[11] has confirmed these observations, apparently establishing this variety of diabetes mellitus, of which a very rapid course--six months to three years--and the habitual presence of diarrhoea are characteristic; while the presence of greasy or creamy stools, and the appearance in them of undigested nitrogenous substances, may aid in the diagnosis. Precisely such a case, running the same rapid course--less than one year--with emaciation, uncontrollable diarrhoea, creamy stools, jaundice, and pancreatic disease, came under the writer's care in 1882. At the autopsy the pancreas was found enlarged, and numerous gritty particles were disseminated through it.

[Footnote 10: "Notes et réflexions à propos de deux cas de diabète sucre avec altération du pancréas," Bull. Acad. de Méd., Paris, 1877, 2d Serie, vi. 1215-1240.]

[Footnote 11: Med. News and Abstract, vol. xxxix., June, 1881, p. 344, from Jour. de Méd. et de Chir. pratiques, Dec, 1880.]

Supposing such pancreatic disease to be primary, it is evident that it must operate through the coeliac plexus, which, with its ganglion, is gradually encroached upon. On the other hand, it is also possible that the disease of the coeliac plexus may be primary, and the coexisting pancreatic disease and diabetes mellitus both secondarily dependent upon it. This can only be settled by more careful study of the coeliac plexus after death from diabetes, but up to the present time facts would seem to support the view of primary pancreatic disease.

The liver is frequently enlarged--sometimes but slightly, at others decidedly. It has been known to reach three times the size of the normal organ. Again, it may be darker and harder--hyperæmic. By minute examination the acini are found enlarged, the capillaries dilated and distended; the liver-cells are enlarged, distinctly nucleated, rounded, and indistinct as to their outline, appearing to fuse into each other. A weak solution of iodine strikes a wine-red color, which, according to Rindfleisch, is confined to the nucleus, but, according to Senator, may extend to the whole cell. This reaction Klebs ascribes to post-mortem changes in the glycogenic substance. They are more striking in the portal or peripheral zone of the lobule, while the intermediate or hepatic artery zone is often fatty, and the central part, surrounded by the rootlets of the hepatic vein, is nearly normal. Stockvis and Frerichs ascribe the enlargement of the liver partially to a new formation of liver-cells--in other words, to a true hypertrophy. At other times the organ has been found reduced in size.

Dickinson, Trousseau, and Budd describe an overgrowth of connective tissue, as well as of the cells of the liver, producing a hypertrophic cirrhosis.

According to Beale, Frerichs, and Folwarczny, the fat which is found in small proportion in the liver-cells in health is often diminished, and even absent, and quantitative[12] analysis by the last-named observer {202} confirms this view. Such diminution may be the forerunner of an atrophy of liver-cells which has been noted, and which, as the disease continues, leads to the atrophy referred to as occasionally present. On the other hand, intense fatty degeneration of the entire organ, similar to that found in phosphorus-poisoning, has been met by Gamgee, associated with a lipæmic state of the blood and symptoms of acute acetonæmia.

[Footnote 12: Folwarczny, "Leberanalysen bei Diabetes Mellitus," Wiener Zeitschr., N. F., 1859, ii. 6.]

The kidneys, in cases which have continued some time, are apt to be hyperæmic and enlarged, although primarily they are uninvolved. It would seem that the long-continued hyperæmia which is a necessary condition of the copious secretion of urine, results, sooner or later, in an over-nutrition of the renal epithelium, a widening of the tubules, and consequent enlargement of the whole organ. The changes are mainly of a parenchymatous or catarrhal rather than an interstitial nature, the epithelium being disposed to shed. These changes may reach a more advanced stage of cellular degeneration, and may be attended by albuminuria. The cells may become very large, present a yellowish-brown color, their nuclei indistinct and non-responsive to ordinary staining solutions, but may take a red stain with a weak solution of iodine, similar to that described in the case of the liver-cells. Mackenzie describes a hyaline degeneration of the intima of the arterioles and a skeleton condition of the epithelium of the collecting tubes.[13] There may also be a catarrh of the pelves of the kidneys and ureters, due to irritation of the saccharine urine.

[Footnote 13: Loc. cit.]

Atrophy of the testes has been noted by Romberg and Seegen in young men, and recently Hofmeier[14] has reported the case of a young diabetic woman, aged twenty, who came under observation for pruritus vulvæ, in whom the uterus was found small, scarcely 5 cm. (2 inches) long, and the ovaries very much atrophied. As this young woman had no other ailment, the atrophy was ascribed to the diabetes.

[Footnote 14: Berliner klin. Wochenschr., 1883, No. 42.]

Among the most constant secondary lesions is the aggregate of changes known as those of pulmonary phthisis. But a few years ago, when our ideas on this subject were more definite than they are to-day, and when it was thought we had three distinct varieties of phthisis--the tubercular, the catarrhal, and the fibroid--the phthisis of diabetes was regarded as typically catarrhal.[15] At the present time, however, when the tendency at least is to regard all phthisis as tubercular, diabetic phthisis must be consigned to the same category. At the same time, if the tubercle bacillus is to be regarded as the essential criterion of tuberculosis, it must be stated that the diabetic patient is subject to two different lung processes--at least if the observations of Riegel of Giessen[16] are to be regarded as correct. In two cases of diabetic phthisis studied at his clinic, the sputum of one contained numerous bacilli, while the other, although the case presented the most distinct signs of infiltration of the apex, and although more than fifty preparations were investigated, revealed none. The sputum was also said to present some unusual physical characters. So far as I know, no autopsies of cases showing these clinical differences have been reported, although there have been found in diabetes, distinct from the usual cheesy foci, fibroid changes with small smooth-walled cavities. In such cases {203} tubercle bacilli would be absent, while the physical signs of consolidation would be present.

[Footnote 15: See the writer's work on Bright's Disease and Diabetes, Philada., 1881, p. 256.]

[Footnote 16: Medical News, Philada., May 19, 1883, from Centralblatt f. klin. Med., Mar. 31, 1883.]

As a part of the phthisical process in diabetes, cavities of various sizes are found and gangrene of the lungs has been observed.

ETIOLOGY.--The problem of the etiology of diabetes mellitus is as unsatisfactorily solved as is that of its pathogenesis. Certainly, a majority of cases of diabetes cannot be accounted for. A certain number may be ascribed to nervous shock, emotion, or mental anxiety; a few to overwork; some to injury and disease of the nervous system; others to abuses in eating and drinking. Among the injuries said to have caused diabetes are blows upon the skull and concussions communicated to the brain, spinal cord, or vaso-motor centres through other parts of the body. Hereditation is held responsible for a certain number of cases. Malarial and continued fevers, gout, rheumatism, cold, and sexual indulgence have all been charged with producing diabetes.

Diabetes mellitus is most common in adult life, although Dickinson reports a case at six years which was fatal, Bence Jones a case aged three and a half, and Roberts another three years old; and in the reports of the Registrar-General of England for the years 1851-60 ten deaths under the age of one and thirty-two under the age of three are included. This statement, in view of the experience of the difficulties of diagnosis in children so young, seems almost incredible. I have never myself met a case in a child under twelve years. At this age I have known two, of which one, a boy, passed from under my notice, while the second, a girl, recovered completely. The disease is most common between the ages of thirty and sixty. The oldest patient I have ever had died of the disease at seventy-two years, having been under my observation for three and a half years.

It is decidedly more frequent in men than in women, carefully prepared statistics of deaths in Philadelphia during the eleven years from 1870 to 1880, inclusive, giving a total of 206 deaths, of which 124, or three-fifths, were males, and 82, or two-fifths, females. This is the experience of all.

My own experience has been singular and interesting. Up to April, 1881, I had never met a case in a woman. Of 18 cases outside of hospital practice which I have noted since that date, 9 were men and 9 women. But I still do not recall an instance of a woman in hospital practice, although I have constantly cases among men.

Not much that is accurate can be said of the geographical distribution of the disease. It seems to be more common in England and Scotland than in this country, at least if the statistics of New York and Philadelphia are considered. In the former city, statistics extending over three and a fourth years show that out of 1379 deaths, 1 was caused by diabetes; in Philadelphia, in eleven years, 1 out of 875; in England and Wales, according to Dickinson from observations extending over ten years, 1 out of 632; and in Scotland, 1 out of 916. According to the same authority, the disease is more prevalent in the agricultural counties of England, and of these the cooler ones, Norfolk, Suffolk, Berkshire, and Huntingdon. According to Senator, it is more common in Normandy in France; rare, statistically, in Holland, Russia, Brazil, and the West Indies, while it is common in India, especially in Ceylon, and relatively very frequent in modern times in Wurtemberg and Thuringia. Seegen says it is more {204} frequent among Jews than among Christians, but I have never seen a case in a Hebrew.

SYMPTOMS, COURSE, AND DURATION.--The earliest symptom commonly noted by the diabetic is a frequency of micturition and the passage of larger amounts of urine than is natural. Coincident with or immediately succeeding this is an undue thirst and dryness of the mouth, which soon becomes the most annoying symptom the patient has, the freest draughts of water giving but partial or temporary relief. To this succeeds dryness, and sometimes itching, of the skin and absence of perspiration. A good appetite with fair digestion accompanies this stage of the disease, but notwithstanding this the patient loses in weight. If a male, his attention is sometimes called to his urine by the white spot left after the evaporation of a drop of urine on his boot or clothing or by the stiffness of his linen due to the same cause. To these symptoms are sometimes added an intolerable itching of the end of the urethra in males and of the vulva in females, probably due to the irritation caused by the saccharine urine in passing over and drying upon these parts.

As the disease progresses muscular weakness supervenes. This, however, comes on at varying periods after the incipient symptoms make their appearance. Sexual inclination grows less. The muscular weakness gradually increases, if the disease is not checked, until the patient can barely walk: he totters in his gait, and reminds one of a case of Duchenne's disease. Even before this he sometimes gives up and goes to bed. Often harassing cough ensues, adding its exhausting effect to that of the essential disease. Percussion and auscultation discover consolidation at one apex or over larger areas of the lungs. Dyspepsia and indigestion replace the good appetite which attended the onset of the symptoms, and all efforts to increase the latter are unavailing. The heart begins to flag, and its action is irregular. It finally ceases to act, and the patient dies suddenly, sometimes unexpectedly. Or coma may supervene before death. This coma, known as diabetic coma, is generally ascribed to the accumulation of acetone or acetone-producing substance in the blood. It is supposed to be a product of the decomposition of the sugar in the blood, and the phenomena resulting from its presence are known as those of acetonæmia. Some further account of it will be given in the section on changes in the urine. It is sometimes recognizable by a fruity odor of the breath, which may even pervade the atmosphere of the room in which the patient lies, and may be recognized on entering. It has been compared to the odor of a room in which apples have been kept, again to sour beer, and again to chloroform.

During all this time the thirst and discomfort arising therefrom, continue, although it sometimes happens that toward the end the quantity of urine and its contained sugar diminish and the urine becomes darker in hue.

Such is the course of a typical case of diabetes mellitus. Other symptoms, less conspicuous, are a lowered temperature of the body, from 1° to 2½° F. or even more; cataract, dilatation of the retinal vessels, intraocular lipæmia, functional derangements of vision, including amblyopia, presbyopia, and loss of accommodating power; and occasionally total blindness from atrophy of the retina may be present. I have known almost total blindness to appear very early in the disease, and {205} subsequently to disappear. Derangements of the other special senses, as impairment of hearing, roaring in the ears, and disorders of smell and taste, also occur. Boils and carbuncles are occasional symptoms; although usually late in occurrence, the former are said to be sometimes the first symptoms recognized. Numerous skin affections may occur. Ulcerated surfaces are slow to heal, and gangrene supervenes sometimes spontaneously, but more often as the result of some trifling injury. It may start from a blister produced by cantharides, although such instances are scarcely frequent enough to justify interference with treatment demanding blisters. More frequently surgical operations do badly. Allied to this tendency is a spongy state of the gums, with recession and excavation, resulting, in asthenic cases, in absorption of the alveolar processes and falling out of the teeth. Eczema of the labia and vicinity in females, and a similar irritation about the meatus urinarius in males, are annoying symptoms. A purulent-looking discharge has been seen issuing from the urethra, in which the spores of penicilium glaucum have been recognized by the microscope.

The term diabetic coma is applied to a form of coma which is apt to occur late in the disease, indeed most frequently to terminate it; while it is also used to indicate a train of nervous symptoms of which coma is the terminal one. To this train of symptoms the word acetonæmia is also applied, and should alone be used, while the term diabetic coma should be restricted to the terminal symptom. The coma, as well as the previous nervous symptoms, is considered due to the accumulation in the blood of a product of the decomposition of sugar, formerly believed to be acetone, but now thought to be an acetone-producing substance, probably aceto-acetic acid. It is likely that in all cases of diabetes a small quantity of this substance exists in the blood, from which it is separated by the kidneys and lungs, while it is only when these channels are insufficient for its removal that it accumulates and produces the symptoms described.

Usually, the coma comes on gradually, deepening until it terminates in death. In other instances it is preceded by various symptoms, including dizziness, drowsiness, cephalalgia, delirium, mania, muscular pains, gastric and intestinal symptoms, including epigastric pain, vomiting--sometimes of blood--and even purging; also dyspnoea, with short, panting respiration like that of an animal with both vagi cut, and a fluctuating pulse-rate which continues until coma is established, after which it remains rapid and small. Both the breath and urine may exhale the peculiar odor of acetone, or it may be absent, and the urine strikes the peculiar burgundy-red reaction with perchloride of iron to be again referred to.

These symptoms may be sudden in their occurrence, whence acute acetonæmia, or they may ensue slowly. Ralfe,[17] who has studied the subject of acetonæmia very thoroughly, has called attention to the parallelism between the phenomena of acute acetonæmia and those of acute yellow atrophy of the liver and of phosphorus-poisoning. The sudden, sharp epigastric pain, with gastric disturbance and vomiting, often of blood; the peculiar panting dyspnoea referred to; the short, {206} noisy delirium, followed almost suddenly by deep coma; the fall in temperature as the nervous symptoms develop; the irregular, and finally rapid, pulse,--are all symptoms common to the two conditions.

[Footnote 17: Clinical Chemistry, 1883, p. 98; also Discussion on Diabetes before Pathological Society of London, Lancet, April 7, 1883, p. 592.]

Although acknowledged to be a grave complication, and the most frequent cause of death in diabetes,[18] yet it does not follow that a fatal termination is inevitable when diabetic coma sets in. I have now a patient, a woman, who considers herself in perfect health, but in whom there remains a trifling glycosuria, who at one time was supposed to be dying of diabetic coma.

[Footnote 18: Of 400 cases of diabetes which passed under the observation of Frerichs, the majority died of acetonæmia (Frerich's "Ueber den plötzlichen Tod und über das Coma bei Diabetes," Zeitschr. für klin. Med., 1883, vi. 3-53). Of 53 persons dying of diabetes at Guy's Hospital, London, during the last ten years, 33 died comatose (Dr. Fred. Taylor, Discussion on Diabetes, Pathological Society of London, Lancet, May 5, 1883). In my own experience acetonæmia has not been so frequent a cause of death as phthisis, acute pneumonia, and heart-failure.]

Crampy pains in the legs and facial paralysis are among the nervous symptoms sometimes present, and the term diabetic neuralgia has been applied to a special form of neuralgia peculiar to this disease. It is characterized by its acuteness, stubbornness, and symmetry. Its favorite seats are the inferior dental nerves and the sciatics. Greisinger referred to the frequency of sciatica in 1859, Braun again in 1868, and others still later; but Worms in 1881 established the close relation between the two conditions and the features described. Most recently (1884), Cornillon[19] collected 22 cases of diabetic neuralgia, and has further elaborated the study. Believing that diabetes affects particularly those persons who have had serious attacks of rheumatism and gout, he is inclined to think the neuralgia as much due to uricæmia as to hyperglycosuria, and that these conditions cause, not neuritis, but transitory lesions in the nerve-centres, but whether in the membranes or gray or white matter is undetermined.

[Footnote 19: "Des nevralgies diabétiques," Revue de Médecine, 1884, iv. 213-230.]

That the phenomena of acetonæmia are those of a toxic agent or agents in the blood derived from the sugar there present is generally conceded, although Sanders and Hamilton,[20] after a study of the clinical histories and the result of autopsies in several cases, are disposed to ascribe diabetic coma to slow carbonic-acid poisoning due to fat embolism of the pulmonary vessels. So far as I know, these conclusions have not been reached by any other observers. R. H. Fitz[21] and Louis Starr[22] have each reported cases of diabetic coma with lipæmia, carefully studied with this point in view, without finding any facts to sustain the carbonic-acid theory.

[Footnote 20: Edinburgh Med. Journal, July, 1872.]

[Footnote 21: "Diabetic Coma; its relations to Acetonæmia and Fat Embolism," Boston Medical and Surgical Journal, vol. cvi. p. 24, Feb. 10, 1881.]

[Footnote 22: "Lipæmia and Fat Embolism in Diabetes Mellitus," New York Medical Record, vol. xvii., 1880, p. 477.]

Alterations in the Blood.--The blood of diabetics is variously charged with sugar, which may be in such quantity as to impart a viscidity and higher specific gravity to the plasma, which has reached 1033, the normal being 1028. On the other hand, analyses have sometimes failed to discover sugar in the blood after death, the result, probably, of the tendency of the sugar to rapid disintegration. Alcohol and acetone, or {207} acetone-producing substance (aceto-acetic acid), are occasionally present as the products of such decomposition, to which are ascribed the symptoms of acetonæmia already discussed.

The presence of fat in the blood of diabetics was noted by the earliest students of the disease. It is sometimes sufficient in amount to produce a milky appearance of the serum, while the analyses of Simon revealed a quantity of 2 to 2.4 per cent., the normal being 1.6 to 1.9 per cent. The fat thus present is said to be sometimes sufficient to cause fat embolism in the capillaries of the lungs, and cases of this condition have been reported by Sanders and Hamilton,[23] Louis Starr,[24] and Rickards.[25] Ralfe ascribes the lactescent appearance of the blood to the action of the aceto-acetic acid, since acetic will give a milky appearance when agitated with a dilute and slightly alkaline mixture of fatty matter at 100°, and the injection of acids into the blood of animals leads to the increase of fatty matter in the blood and fatty infiltration of tissues.

[Footnote 23: Loc. cit.]

[Footnote 24: Loc. cit.]

[Footnote 25: Birmingham Med. Review, Jan., 1882.]

It must be admitted that the mode in which this lipæmic state of the blood is brought about is imperfectly understood, and whether it be by some chemical agency of the kind described by Ralfe, or by rapid absorption of the subcutaneous fat, or from an imperfect oxidation of absorbed fat, is undetermined. Possibly all may contribute.

Albert G. Heyl[26] has described an altered appearance of the retinal vessels recognizable by the ophthalmoscope, which he ascribes to the fatty blood-plasma at the periphery of the blood-current, the normal plasma being invisible on account of its transparency.

[Footnote 26: For a detailed description of this appearance, with a colored lithograph depicting it, see the author's work on Bright's Disease and Diabetes, p. 262.]

The red blood-discs are diminished and their ratio to the white corpuscles altered. In a count by F. P. Henry, in Louis Starr's case, the number of red discs was 4,205,000 to a cubic millimeter, the normal being at least 5,000,000; the white were 50,000 to a cubic millimeter, or 1 white to 84 red, instead of 1 to 350 or 500.

Changes in the Urine.--The most important changes in the urine are its increase in quantity and the presence of sugar. The variations in the former are extreme, being from an amount which but slightly exceeds the normal to as much as 50 pints (23.65 liters) in twenty-four hours, and even more. The quantity is of course limited by the fluid ingested, and although it may exceed this amount for a day or more, it cannot do so for any length of time. It is generally a little less. The more usual quantity in the twenty-four hours is from 70 to 100 ounces (210 to 300 cc.).

The quantity of sugar varies greatly in different cases and at different times in the same case. The maximum quantity reported by Dickinson was 50 ounces, or 1500 grammes, in twenty-four hours. The proportion may reach as much as 15 per cent., but the more usual amounts are from 1 to 8 per cent., or from 5 to 50 grains (.324 to 3.24 grams) to the fluidounce, or from 300 to 4000 grains (19.44 to 260 grams) in the twenty-four hours.

It is important to know that intercurrent febrile disease may produce a decided diminution in the daily quantity of urine, and of the sugar contained in it. A similar decrease, and even disappearance, is said to take place sometimes toward the fatal termination of a case.

{208} The effect of exercise upon the sugar secretion is not uniform. Bouchardat and Kuelz have noted a diminution, and even disappearance, of sugar from urine as its result, and it is reasonable to suppose that judicious exercise is at least without harmful effect, while it is certain too that muscular exercise, if excessive, will increase glycosuria.

Changes in diet of course modify the secretion of sugar, starches and saccharine foods increasing it, while nitrogenous and oily foods diminish it. So, too, the urine secreted on rising in the morning has almost always less sugar in it than that passed on retiring; and it is not rare to find no sugar in urine passed on rising, when that passed on retiring at night may contain a small amount of sugar--from ¼ to 1 per cent. On the other hand, I have found a small amount of sugar in the morning urine when the evening urine contained none. Anxiety and excitement both increase the proportion of sugar.

Inosite, or muscle-sugar, is sometimes associated in urine with diabetic sugar, and occasionally replaces it. So, too, in experiments upon animals puncture of the fourth ventricle is sometimes followed by inosuria instead of glycosuria, and in corresponding organic disease of the brain the same thing is observed. The substitution of grape-sugar by inosite in the course of diabetes is considered by Laboulbène[27] a favorable change.

[Footnote 27: "Note sur l'Inosurie, succédant au diabète glycosurique, et paraissant avoir une action favorable," L'Union Médicale, Oct. 14, 1883.]

As would be expected, the specific gravity of saccharine urine is usually high--most frequently from 1025 to 1040--and Bouchardat noted a specific gravity of 1074 in one instance. On the other hand, I have found sugar easily detectable in urine with a specific gravity as low as 1010. Pavy records an instance of the same specific gravity, and Dickinson one in which the specific gravity was as low as 1008. It is to be remembered that the sugar is rapidly destroyed when fermentation sets in. A coincident diminution in the urea and other solids of the urine will reduce the specific gravity of a saccharine urine otherwise heavier.

The depth of color of diabetic urine is inversely as the quantity passed. Hence, when this is very large the urine is pale, and even almost colorless, but it may still contain considerable amounts of sugar and possess a decided color, quite as deep as that of urine passed in smaller quantity. When exposed to the air, diabetic urine becomes rapidly turbid from the growth of fungi, including the yeast fungus and penicilium glaucum.

The odor of diabetic urine just passed is usually in no way peculiar, but as fermentation progresses an acetous odor is developed, which is ascribed to acetic acid. At other times the odor is quite peculiar, being spoken of as vinous or compared to that of sour beer, stale fruit, alcohol, chloroform, or, as by one of my patients, to sweetbrier.

Diabetic urine has almost invariably an acid reaction, which becomes more decided as fermentation progresses. As a consequence of this increased acidity, and sometimes independent of fermentation-changes, the urine deposits a sediment of uric acid, but with this exception diabetic urine is generally free from sediment. Diabetic patients on a meat diet sometimes have a good deal of uric acid from this source.

Albuminuria may coexist with glycosuria, but is not generally found until late in the disease, after changes in the kidney begin to make their {209} appearance, unless, as may happen, glycosuria supervenes upon primary renal disease.

Alcohol and acetone, or an acetone-yielding substance--aceto-acetic acid--are sometimes found in diabetic urine. They are products of the breaking up of sugar, but chemists do not explicitly agree as to the exact method in which acetone originates in the organism. First recognized in the distillate of urine and blood of a diabetic patient by Petters[28] through its physical properties, odor, combustibility, etc., rather than by actual isolation, it was further investigated by Kaulich,[29] Gerhardt,[30] Rupstein,[31] and Markownikoff,[32] who obtained it in an impure state from urine; by Deichmüller and Tollens,[33] whose isolated substance was pure, and finally most recently by Jaksch[34] and Penzoldt.[35] The former found it not only in diabetic urine, but also in that of fever, and even of carcinoma. The latter found it by the indigo test in but 18 out of 22 diabetics, and by the iodoform test, either decidedly or feebly, in 20 out of 20; in 3 out of 11 cases of typhoid fever, in 6 out of 7 cases of pneumonia, in none of 6 cases of phthisis, in 1 out of 3 cases of measles, and in 1 case of cerebro-spinal meningitis. Finally, v. Jaksch has been led to believe, from his extensive investigations, that acetone is a constant and normal product of tissue-change, although Penzoldt considers such conclusion scarcely justified.

[Footnote 28: Prager Vierteljahrschrift, xiv. 3, 1857, S. 88.]

[Footnote 29: Ibid., xvii. 3, 1860, S. 59.]

[Footnote 30: Wiener Med. Presse, No. 28, 1865.]

[Footnote 31: Centralbl. für d. med. Wiss., No. 55, 1874.]

[Footnote 32: Liebig's Annalen, Bd. 182, S. 362.]

[Footnote 33: Ibid., Bd. 209, S. 25.]

[Footnote 34: Zeitschrift für physiol. Chemie, vi. 6.]

[Footnote 35: "Beiträge zur Lehre von der Acetonurie und von verwandten Erscheinungen," Deutsch. Archiv für klin. Med., xxxiv., 2 Oct., 1883, S. 127.]

Gerhardt early discovered a substance in the urine of diabetics and habitual drinkers which struck a deep-red reaction with chloride of iron. This he considered was the source of acetone, and was probably ethyl diacetate or diacetic ether, which by decomposition yields equal molecules of acetone and alcohol; thus:

C_{4}H_{5}O_{3}C_{2}H_{5} + H_{2}O = C_{3}H_{6}O + CO_{2} + C_{2}H_{6}O.

Ethyl diacetate. Water. Acetone. Alcohol.

This view is still held by some, but others, in view of the recent discovery of Deichmüller and Tollens,[36] that diabetic urine when distilled yields decidedly more acetone than alcohol, have suggested that the substance is derived from aceto-acetic acid.

[Footnote 36: Loc. cit.]

The first test suggested for acetone was Gerhardt's chloride-of-iron test. A solution of chloride of iron added to urine containing acetone strikes a burgundy-red color. But this reaction occurs with so many substances that it cannot be considered entirely reliable. Ralfe's modification of Lieben's iodoform test[37] is made as follows: About a fluidrachm (3.7 c.c.) of liquor potassæ, containing 20 grains (1.2 grams) of iodide of potassium, is placed in a test-tube and boiled; a drachm (3.7 c.c.) of the suspected urine is then carefully floated upon the surface. When the urine comes in contact with the hot alkaline solution a ring of phosphates is formed, and after a few minutes, if acetone or its allies are present, the ring will become yellow and studded with yellow dots of iodoform, which, in turn, will sink through the ring of phosphates and deposit itself at the bottom of the test-tube. A number of other substances {210} produce the iodoform reaction, but only one of these, lactic acid, is likely to be met in urine.

[Footnote 37: Clinical Chemistry, Philadelphia, 1884, p. 100.]

The perspiration, saliva, exudations, and effusions in diabetic cases have all been found, at times, to contain sugar.

DURATION.--Diabetes is a disease of which the duration is measured by months and years, and although cases are reported in which death supervened in from six days to six weeks after the recognition of the disease, it is evident that such periods do not necessarily measure its actual duration. The disease may have existed some time before coming under observation. On the other hand, a case is reported by Lebert which lasted eighteen years; another, under the successive observation of Prout and Bence Jones, sixteen years; and a third, under Bence Jones and Dickinson, fifteen years. The younger the patient the shorter usually is the course run and the earlier the fatal termination. Yet I have known a girl of twelve recover completely. After middle age the disease is usually so easily controlled by suitable dietetic measures, if the patient is willing to submit to them, that its duration is only limited by that of an ordinary life, while carelessness in this respect is apt to be followed by early grave consequences.

COMPLICATIONS.--The almost sole complication of diabetes mellitus is the tubercular phthisis which so often terminates it. Indeed, it is doubtful whether this complication should not be regarded as a consequence, as should also the boils, gangrenous processes, and ophthalmic conditions which have been mentioned under Symptomatology. Jaundice has occurred three times in my experience up to the present time. Senator says that when not an accidental complication due to a catarrh of the duodenum it may result from compression of the biliary capillaries by the overloaded blood-vessels and enlarged gland-cells of the liver. In one of my cases, in which jaundice appeared to be the initial symptom, but which disappeared some months before death, the autopsy revealed atrophy of the liver. It is well known that pancreatic disease, especially cancer, is apt to be accompanied by jaundice, and as pancreatic disease is often at the bottom of diabetes, it will similarly account for the jaundice, while the presence of jaundice may also suggest a pancreatic diabetes.

DIAGNOSIS, INCLUDING THE TESTS FOR SUGAR IN THE URINE.--The diagnosis of diabetes mellitus, the disease being once suspected, is easy. The passage of large amounts of pale urine of high specific gravity, the presence of thirst, dryness of the mouth, fauces, and skin, and progressive emaciation even while the appetite is good, can scarcely be misinterpreted. In the urine from such a case the application of any of the tests for sugar will produce prompt response. The urine is not always so much increased as to attract attention, while its color is also sometimes but slightly changed; but the symptoms of thirst and dryness or clamminess of the mouth are seldom wanting. On the other hand, the discovery of a glycosuria without these symptoms is, as a rule, accidental. It is a question how far such degrees of glycosuria as do not produce the usual symptoms of diabetes in an appreciable degree are signs of positive disease. At the same time, its detection is important, in that there is always danger of the simple glycosuria becoming a diabetes--a danger which its recognition and suitable treatment may avert. Accordingly, the urine of all persons having unusual appetites without evident cause, {211} and of those who are fond of eating and drinking, should be tested for sugar. This should also be done for those who have passed through severe mental or physical strain, have suffered shock or concussion of the nervous system, blows upon the abdomen, etc.

Testing for Sugar.--Under the head of Diagnosis I prefer to include the testing for sugar, which requires some detailed consideration. Unless it be that the indigo test recently revived by George Oliver of London prove more delicate, that form of cupric test known as Fehling's solution is, with suitable precautions, all things considered, the most satisfactory for general use.

Fehling's volumetric solution, suitable for both qualitative and quantitative purposes, is made as follows: Dissolve 34.639 grams of pure crystallized cupric sulphate in about 200 cubic centimeters of distilled water; 173 grams of chemically pure crystallized neutral sodio-potassic tartrate and 80 grams of potassium hydrate in 500 or 600 c.c. of distilled water. To the latter add the copper solution slowly, and dilute the clear mixed fluid to 1 liter. One cubic centimeter of this solution will be decolorized by 0.005 grm. of sugar, or 200 grains will be decolorized by 1 grain of sugar. Or the copper may be dissolved in 1 liter of water, and the tartrate and potassium hydrate in another, and a cubic centimeter of each mixed at the moment they are to be used.

For qualitative testing, put a cubic centimeter of Fehling's solution into a test-tube (or if the copper and the alkaline sodio-potassium tartrate solutions are kept separate, a cubic centimeter of each), and dilute with distilled water to 5 c.c. Boil, and if, after the lapse of a couple of minutes, the solution remain unchanged, it is fit for testing. If it becomes turbid or a red sediment falls, it is spoiled, and a new solution should be obtained.[38] A cubic centimeter of the suspected urine is then measured out and added drop by drop to the solution kept hot. If there is much sugar, the first drop will throw down a yellow precipitate of suboxide of copper, which becomes rapidly red. If no reaction takes place after adding the entire cubic centimeter of urine, the addition should be continued until 4 c.c. are added, when, if, after the mixture has cooled, there be no response, it may be concluded that the urine is free from sugar. By operating with a cubic centimeter of the test-fluid and the same quantity of urine or multiples thereof, we may roughly estimate the proportion of sugar. Thus, if the cubic centimeter of undiluted urine just decolorizes the cubic centimeter of Fehling's solution, sugar is present in the proportion of one-half of 1 per cent.; or if a half cubic centimeter of the urine removes all the color, the quantity is 1 per cent. If the urine is highly charged with sugar, it may be diluted, and the degree of dilution being remembered, a rough quantitative estimation may be similarly made.

[Footnote 38: Should this not be possible, a little more soda may be added and the fluid filtered, when it is again ready for use.]

If the urine contains very minute quantities of sugar, the reaction is less satisfactory. The copper is reduced, but the suboxide is so small in quantity that it is obscured by the excess of copper solution, and a mixture results which is greenish or greenish-yellow or yellow or milky, and on standing a small yellow sediment falls to the bottom. Now, it dare not be said that it is sugar which produces such reaction. It may be {212} sugar, but it may also be uric acid. Uric acid is really more frequently a source of error than is commonly supposed. I have myself seen the reaction due to it so vivid that I did not suspect it could be due to any reducing agent excepting sugar; but, noting the next day a copious sediment of uric acid which had fallen during the night, a testing of the supernatant fluid then revealed no reaction whatever. Such a urine, after being treated by the lead process to get rid of the uric acid, fails also to respond. But this process is very tedious,[39] and cannot be conveniently carried out by the busy practitioner. The same thing is, however, accomplished by treating the urine with hydrochloric acid, which in twenty-four hours precipitates all of the uric acid. Simple precipitation by lead acetate solution and filtration does not answer, because all of the uric acid is not thus removed. Other substances, as hippuric acid, urates, hypoxanthin, etc., are said to act similarly, but they produce no practical interference with the test. On the other hand, a small amount of sugar may be present and yet fail to show the reaction, because the cuprous oxide is held in solution by certain substances. Such are ammonia and nitrogenous matters, including albumen, creatinin, pepsin, peptones, urinary coloring matters, etc. The latter probably produce their effect through the ammonia which is given off while heating them in the presence of an alkali. Hence all albumen should be precipitated and filtered out of urines suspected to contain sugar, and the heat applied should not be too great. Finally, excess of glucose will also hold in solution cuprous oxide, so that the suspected urine should not be added in too large a quantity at a time, but rather drop by drop.

[Footnote 39: The details of this process will be found in the writer's work on the Practical Examination of Urine, 5th ed., 1883, p. 63.]

But qualitative testing is not sufficient during the treatment of a case of diabetes. The percentage of sugar and the quantity discharged in twenty-four hours should be determined occasionally. The process is done as follows: Place 10 cubic centimeters of Fehling's solution in a porcelain capsule, and dilute it with 40 c.c. of distilled water. Fill a Mohr's burette with the urine, which, if it contain more than 1 per cent. of sugar, should be diluted with nine times its bulk of distilled water. Slowly heat the contents of the capsule to boiling, and then allow a little of the diluted urine to run in from the burette; continue the cautious addition of urine and the gentle heating until the blue color is completely removed from the Fehling's solution. To determine the exact moment at which this takes place requires a little experience, but its recognition is facilitated by carefully tilting the capsule after each addition and stirring, so that its clear white surface may be seen through the edge of the fluid and contrasted with the latter. The number of cubic centimeters of urine used should now be read off from the burette, the number of c.c. of undiluted urine calculated therefrom, and each c.c. multiplied by .005 grm. The result indicates the quantity of sugar in grams in the urine employed, whence the percentage of sugar is determined, and also the twenty-four hours' quantity, the amount of urine passed in that period being known.

The Fermentation Test.--A very simple and easy method of determining the proportion of sugar is by Roberts's fermentation method, which, although not so precise as the volumetric process, is still {213} sufficiently so for clinical purposes. A small piece of German yeast or a teaspoonful of liquid yeast is added to about four ounces (120 c.c.) of the urine, which is kept lightly stopped, at a temperature of 20° to 30° C. (68° to 80° F.), for about twelve hours; at the end of this time the sugar will have been converted into alcohol and carbonic acid. The latter will have passed off, and the urine lost in weight because of the destruction of sugar; while the difference between the specific gravity before and after the fermentation indicates the number of grains of sugar per fluidounce. Thus, suppose the specific gravity before fermentation to have been 1040, and afterward 1025; there will have been 15 grains of sugar to the fluidounce, whence, again, the twenty-four hours' quantity can be calculated. If the metric system is used, each degree of specific gravity lost will correspond to .2196 grams of sugar in every 100 c.c. of urine.

The specific gravity of the fermented urine should be compared with that of the urine soon after it is passed, because saccharine urine under suitable circumstances undergoes fermentation without the addition of yeast; and, the specific gravity being thus lowered spontaneously, the reduction in the urine fermented by yeast would appear less than it actually is. At the same time, care should be taken that the urine is of the same temperature when the specific gravity is taken before and after fermentation.

The Picric Acid and Potash Test.--Although attention was called in 1865 by C. D. Braun,[40] a German chemist, to a reaction between grape-sugar and picric acid, as the result of which the latter is converted into picramic acid, very little attention seems to have been paid to this announcement. Quite ignorant of it, George Johnson rediscovered this reaction in 1882, and published it in 1883.[41] It is applicable to both qualitative and quantitative purposes. In order to make use of it, a standard comparison-solution is made as follows: Take 1 fluidrachm of a solution of grape-sugar, 1 grain to the fluidounce; mix it in a long test-tube with half a drachm of liquor potassæ (U. S. P. or B. P.) and ten minims of a saturated solution of picric acid; dilute the mixture to 4 fluidrachms with distilled water, to facilitate which a tube used for the purpose may be marked at 4 fluidrachms. Raise the mixture to the boiling-point, and continue the boiling for sixty seconds, to ensure complete reaction between the sugar and picric acid. During the boiling the pale-yellow color of the liquid is changed to a vivid claret-red. Cool the liquid by cautiously immersing the tube in cold water, and if it is not then at the level of the 4-drachm mark, raise it to this by adding distilled water. The standard color thus obtained is that which results from the decomposition of picric acid by a grain of sugar to the ounce, four times diluted, or by a solution of sugar containing one-quarter of a grain per ounce. But the picramic solution rapidly becomes pale on exposure, so it becomes necessary to make a more permanent solution to use as a standard. This may be accomplished by combining liquor ferri perchloridi drachm j, liquor ammonii acetatis drachms iv, acidum aceticum (glacial) drachms iv, and water enough to make ounces iiss. The color of this is identical with that of the picric acid reduced by a one-grain solution diluted four times, and, {214} according to Johnson, it will retain its color unchanged for at least six months. At the same time, whenever a new solution is made it should be compared with that of the one-quarter grain per ounce solution of sugar, boiled with picric acid and potash.

[Footnote 40: "Ueber die Umwandlung der Pikrinsaüre in Pikramminsaüre, und Ueber die Nachweisung der Traubenzucker," Zeitschrift für Chemie, 1865.]

[Footnote 41: British Medical Journal, March, 1883.]

For qualitative testing Johnson directs: To a drachm of urine in a test-tube add a few drops, enough to give a distinct yellow color, of a saturated solution of picric acid. Add about 10 drops of liquor potassæ and boil. If sugar is present, the mixture becomes promptly red in hue.

The quantitative estimation is based upon an accurate approximation, by dilution, of the color of the tested fluid with that of the standard solution. Johnson recommends the picro-saccharimeter figured in the text. This is a stoppered tube twelve inches long and three-quarters of an inch in diameter, graduated into ten, and each of these again into ten other equal divisions. By the side of this tube, and held in position by an S-shaped band of metal, is a stoppered tube of equal diameter and about six inches long, containing the standard solution corresponding to the reaction of the one grain of grape-sugar with picric acid and potash diluted four times.

It has been found that ten minims of a cold saturated solution of picric acid are rather more than sufficient for decomposition by one drachm of a solution of grape-sugar in the proportion of one grain to the ounce. A drachm of the solution will therefore contain one-eighth of a grain of sugar, which is the strength of the solution used in making the standard-color liquid. In making the analysis, while the quantity of liquor potassæ used is always the same and the dilution is always to four drachms, the picric acid must be added in proportion to the amount of sugar present, so that if the urine contains as much as six grains to the fluidounce, sixty drops or a fluidrachm of the picric-acid solution would have to be used; and when the proportion of sugar is higher than this, the urine should be diluted with distilled water five or ten times before commencing the analysis, and the degree of dilution remembered in the computation.

If, now, a drachm of a solution of grape-sugar, containing two grains to the ounce, be mixed with the same quantity of liquor potassæ and picric acid and increased by the addition of distilled water to four drachms in the boiling tube, and boiled as before for sixty seconds, the result will be a mixture of much darker color than will be produced by the one-grain solution; but if the dark liquid be diluted with its own volume of water, the color will be the same as that of the one-grain solution or the standard.

It is plain, then, that if a given quantity of the dark saccharine fluid produced by boiling--say, enough to cover ten divisions of the graduated tube, as shown in the figure--has to have added to it an equal bulk of distilled water in order to produce {215} the color of the standard solution, the tested fluid will be of the strength of two grains to the ounce; if three times, three grains; and so on; while fractional additions, as indicated by the graduated markings, would show fractional additions to the proportion of sugar.[42]

[Footnote 42: A more exact comparison of the saccharine liquid with the standard is made by pouring into a flat-bottomed colorless tube six inches long and an inch in diameter as much of the standard solution as will form a column about an inch in height, and an exactly equal column of the saccharine fluid in a precisely similar tube. The operator then looks down through the two tubes at once, one being held in each hand, upon the surface of a white porcelain slab or piece of white paper. In this way slight differences of tint are easily recognized; and if the liquid to be analyzed is found darker than the standard, it is returned to the graduated tube and diluted until the two liquids are found to be identical in color, when the final reading is made.]

The presence of albumen, even in considerable amount, has but little effect upon the test, nor does the coloring matter of normal urine, according to Johnson; but he says there is a coloring matter associated with ser-albumen in albuminous urine, and with egg-albumen as well, which has a reducing action on picric acid. This is partly separated by filtering off the precipitated albumen, and entirely removed by repeated filtration through animal charcoal. So, too, the albumen removed by coagulation and filtration, if thoroughly washed, does not give any red reaction if boiled with picric acid and potash diluted in the same proportion as when testing for sugar. Neither do any other unoxidized sulphur compounds found in urine decompose the picric acid and render the test fallacious.

Johnson and his son, G. Stillingfleet Johnson, claim that the picric-acid test is as accurate as any other, and that it is even more accurate than either Fehling's or Pavy's process, because the picric acid is not acted upon by uric acid or urates, which do reduce the oxide of copper. The method of analysis by the picro-saccharimeter, they claim, is at least as speedy and as easy as any other. The materials and apparatus required are easily prepared, inexpensive, and not, like Fehling's copper solution, liable to undergo rapid changes.

But while Johnson claims that neither coloring matters of normal urine nor uric acid reduce the picric acid, he admits that he has tested with picric acid and potash a large number of specimens of normal urine with the almost uniform result of a depth of color indicating the proportion of .6 of a grain of sugar to the fluidounce, the indication varying between the limits of .5 to .7 grain. The ammonio-cupric method used at the same time gave results of from .7 to .9 grain to the fluidounce, or an excess of .1 to .3 grain. Now, if my own views, the grounds for which are announced elsewhere,[43] are correct, strictly normal urine contains no sugar, and any reducing action upon oxide of copper is due to uric acid, either picric acid is reduced to a degree by uric acid or by some other constituent of normal urine. This, in the light of Oliver's[44] recent investigations, may be kreatinin. For he has shown that kreatinin strikes in a few seconds a red color with the cold alkaline picric solution, which is quickened by heat. From this it would seem that the exact value of the picric-acid test has as yet to be determined.

[Footnote 43: Tyson, Practical Examination of Urine, 4th ed., Philadelphia, 1884.]

[Footnote 44: On Bedside Urine-Testing, including Qualitative Albumen and Sugar, by Geo. Oliver, M.D., London, Member of the Royal College of Physicians of Lond., etc., 2d ed., London, 1884.]

{216} The Indigo-Carmine Test.--The fact that indigotine, the coloring matter of commercial indigo, is converted into indigo when heated with an alkali in the presence of glucose and certain carbohydrates, has recently been applied by George Oliver of London in the construction of a test-paper. Carmine of indigo is the sulph-indigotate of sodium, an intensely blue salt, soluble in 120 parts of water. Sulph-indigotic acid is made by heating indigo with sulphuric acid, and when combined with a base, sodium, produces indigo-carmine. When sodium carbonate is mixed with a solution of indigo-carmine, the latter is precipitated in a minute state of division, but is redissolved on heating, when there results a greenish-blue solution. A freshly-made mixture of the indigo solution and sodium carbonate furnishes a fluid not unlike Fehling's solution, which gives the reaction to be described with glucose. Unfortunately, such a mixture will not keep, and the reagent would be useless but for the happy idea of Oliver of making the test-paper. In doing this bibulous paper is immersed in a solution of indigo-carmine with carbonate of sodium.[45] The paper is then cut into strips an inch long and one-quarter of an inch wide.

[Footnote 45: No more precise directions than this are given by Oliver, either in his papers in the Lancet for 1883 or in his little book just published, On Bedside Urine-Testing. The sugar test-papers, as well as the entire series of albumen test-papers, suggested by Oliver, are now made by Parke, Davis & Co. of New York, and by Wilson & Son, Harrogate, London.]

Mode of Testing.--One of the test-papers and a sodium carbonate paper[46] are dropped into a half-inch test-tube, and water added until the upper end is just covered; a column of fluid one inch in height and half an inch in diameter will thus be produced, so that the solution of carmine obtained on boiling will always acquire the same concentration. Heat is now applied, the tube being gently shaken, and boiling kept up for a second or two. A beautiful blue solution will result. The test-paper may now be removed or allowed to remain.

[Footnote 46: Test-papers of the same size, charged with a saturated solution of sodium carbonate.]

Not more than one drop of the suspected urine is let fall into the tube from a pipette held in an upright position. Drops of equal size are thus secured. The contents of the tube are again freely boiled for a few seconds, after which the tube should be raised an inch or more from the flame and held without shaking, while the solution is kept quite hot, but not boiling, for exactly one minute. If glucose be present in abnormal amount, the soft rich blue will be seen first of all to darken into violet; then, according to the quantity of sugar, there will appear in succession, purple, red, reddish-yellow, and finally straw-yellow. When the last-named color has been developed the slightest shaking of the tube will cause red streaks to fall from the surface and mingle with the pale yellowness of the solution, while further agitation will cause the return of purple and violet and the restoration of the original blue.

The time required for the commencement of the reaction after the boiling of the test liquid is in inverse proportion to the amount of glucose present. When the latter is large, over 20 grains to the ounce, it will be but a few seconds; but when small, 2 or 3 grains, from thirty to sixty seconds may elapse. If the urine do not contain more than the normal amount of sugar[47]--i.e. under half a grain to the ounce--the color of the solution {217} at the end of the heating for one minute will be unchanged. The test is available by artificial light as well as by daylight.

[Footnote 47: It will be noted from this that Oliver accepts the view that there is a small amount of sugar in normal urine.]

Precautions.--1. Care should be taken during the testing not to shake the tube or to permit free ebullition. 2. While keeping the contents of the tube hot, the latter should not be held up between the eye and the sky, for then the early color-changes will probably escape observation. The tube should be kept below the eye-level and its contents viewed by the reflected light of some bright object, such as a sheet of white paper propped up an inch or two beyond the tube as a background. 3. Oliver is not aware that the presence of earthy carbonates will prevent the carmine reaction, but as a precautionary measure he suggests the use of a soda-paper whenever the water is exceptionally hard. 4. The acids of the urine rob the carmine-paper of much alkali, so that the addition of more than a certain number of drops of urine--varying of course with the degree of acidity--will at first retard and then prevent the reaction. The addition of the soda-paper will prevent any such interference, although Oliver says that by invariably submitting only one drop of saccharine urine to the test-paper, and keeping up the heating for not less than two minutes, he has never failed to obtain the characteristic reaction without using a soda-paper. It is well to remember, however, that an excessively acid urine may thus interfere, and that the soda-paper will prevent it. 5. The blue color of the carmine is discharged by caustic alkali--liquor potassæ or sodæ. The only chance of being misled by this reaction lies in using an imperfectly cleansed test-tube which may have contained Fehling's solution or the alkaline picric solution. The caustic alkali converts the blue carmine into a green solution, which, on heating, disappears; nor does it return by again shaking the contents of the tube.

Critical comparison of this test with Fehling's solution and picric acid by Oliver has shown that of sixty-four substances experimented upon, normal and abnormal constituents of urine or medicines which after ingestion are eliminated in the urine, Fehling's was reduced by fifteen, picric acid by eleven, and indigo-carmine by eight. The only substances producing the characteristic play of colors with indigo-carmine test-papers reacted with both picric acid and Fehling's solution. They were unoxidized phosphorus, ammonium sulphide, milk-sugar, dextrin, inosit, gallic acid, tannic acid, and iron sulphate. Both the carmine and picric acid were reduced by inosit, which merely turned Fehling's solution green. On the other hand, uric acid and urates, which reduce Fehling's solution, do not react with the carmine test, while kreatinin, which reacts with picric acid also, does not respond to the carmine. Albumen, if abundant, interferes with Fehling, but not with the indigo-carmine.

Detection of Inosit.--It has been said that inosit sometimes accompanies, and even substitutes, grape-sugar in the course of diabetes. It has been mentioned that it does not reduce Fehling's solution, but turns it olive-green. It reduces the carmine and alkaline picric acid solution, and is therefore not recognizable by these. The methods recommended for its recognition in the books are troublesome, and as its presence in the absence of sugar indicates a favorable change, it is not likely that a more precise recognition than is furnished by the olive-green reaction will be needed for clinical purposes.

PROGNOSIS.--The prognosis in diabetes depends upon the organ whose {218} involvement is responsible for the symptoms, upon the stage at which the condition comes under observation, and upon the age of the patient. It has appeared to me that the cases of diabetes depending upon pancreatic disease are the most intractable, that their progress is scarcely checked by treatment, and that they are comparatively rapidly fatal in their termination. In the others, where the symptom is one of a central nervous lesion, it has always seemed to me to be of secondary importance that the glycosuria is itself less marked, that it is unattended by the other distinctive symptoms of diabetes, and that its issue is that of the nervous malady.

Again, it is well known that the later in life diabetes occurs the more amenable it is to treatment, and that if a proper diabetic diet be adhered to by the patient his life need scarcely be shortened. On the other hand, diabetes mellitus is a disease in which the expectant plan is dangerous. If it does not improve it usually gets worse; and many a patient has fallen a victim to his own indifference and indisposition to adhere to a regimen under which he could have lived his natural term of life. This is especially the case when the disease appears after middle life.

If, on the other hand, the condition becomes thoroughly established before twenty-five years of age, it is less amenable to treatment; but even in such cases a promptly vigorous treatment is sometimes followed by recovery. I have already mentioned the case of a child twelve years old in which complete recovery took place.

If tubercular phthisis supervenes, recovery is not to be expected, while intercurrent disease, as pneumonia, which is rather prone to occur, is very much more serious and apt to terminate fatally.

TREATMENT.--The treatment of the aggregate of symptoms known as diabetes mellitus is conveniently divided into the dietetic, the medicinal, and the hygienic, of which the first is by far the most important. The efficiency of this treatment depends upon the successful elimination from the diet of all articles containing grape-sugar, cane-sugar, beetroot-sugar, and starch, it being universally recognized that in the early stages of the disease these foods are the sole source of the glucose in the urine. The normal assimilative action of the liver, by which the carbohydrates are first stored up as glycogen, and then gradually given out as glucose or maltose to be oxidized, being deranged, such foods not only become useless as aliments, but if continued seem to aggravate the glycosuria, and the excretion of sugar steadily increases. There is, therefore, a double reason for excluding them from the food. This is easiest accomplished by an exclusive milk diet. The exclusive milk treatment of diabetes was suggested by A. Scott Donkin in 1868. That he is correct in his assertion that in the early stages of diabetes lactin or sugar of milk is quite assimilable, and does not in the slightest degree contribute to the production of glycosuria, I cannot doubt; that it is in this respect even superior to casein, as claimed by Donkin, I am not prepared to state from actual knowledge; but that casein itself resists the sugar-forming progress immeasurably greater than any other albuminous substance, so that in all but the most sure and advanced or complicated cases its arrest is complete, I am also satisfied. Certain it is that in a large number of diabetics the use of a pure skim-milk regimen results in a total disappearance of the sugar from the urine. That in a certain proportion of these cases a {219} gradual substitution of the articles of a mixed diet may be resumed without a return of the symptoms is also true. In other more confirmed cases the use of skim-milk results in a decided reduction in the amount of sugar, with an abatement of other symptoms, which continues as long as the diet is rigidly observed. In still other cases, while the skim-milk treatment makes a decided impression upon the quantity of sugar, it still remains present in considerable amount, while the disease progresses gradually to an unfavorable issue. These three classes of cases represent, ordinarily, different stages of the disease, so that it may be said that as a rule cases recognized sufficiently early may be successfully treated with skim-milk, although it may occasionally happen that cases pursue a downward course from the very beginning despite all treatment. Yet I have never seen a case which, when taken in hand when a few grains of sugar only to the ounce were present, failed to yield to this treatment.

As to the method of administration, my practice with adults is to give eight ounces (an ordinary tumblerful) every two hours, beginning at seven or eight o'clock in the morning, and continuing to the same hour in the evening. Sometimes it is well to begin with half as much at first, but rapidly to increase to the required amount. This method ensures the ingestion of three to four quarts daily--a quantity generally sufficient to maintain the body-weight of an adult person of average size and taking moderate exercise, although a slight reduction may take place at first. But if the individual is very active or of large size, it will not be found sufficient. In such event the quantity must be increased as demanded by a feeling of unsatisfied hunger. I have known fourteen pints to be taken in twenty-four hours. But when the quantity becomes thus large, the inconvenience in ingesting it is very great, and it is much more convenient to coagulate the casein of a part of the milk and use the curd thus obtained, while the second part is drunk. Curd may be seasoned with salt to make it more palatable, and should be thoroughly masticated before it is swallowed.

The milk should not be taken too cold, especially if the amount ingested is large, else it is likely to reduce the temperature of the stomach below the point necessary for gastric digestion. The temperature should not be less than 60° F., nor much over 100°. Something depends upon the idiosyncrasies of the patient, which must be the guide as to temperatures intermediate between those named.

The chief advantage of the skim-milk over the unskimmed is simply that it is more easy of digestion. Many persons who cannot take unskimmed milk for any length of time without its deranging the digestion, or, as is commonly said, making them bilious, can take with impunity milk from which the cream is removed. Although Salomon[48] claims to have shown that glycogen is produced in the liver of rabbits fed upon pure olive oil, it is at least probable that fat is among the last of the substances undergoing this conversion, and in ordinary cases of diabetes it is rather its indigestible nature which renders it prudent to remove from milk the greater proportion of fat by skimming it off.

[Footnote 48: Virchow's Archiv, Bd. 61, Heft 3, 1874, 18.]

Still more easily assimilable is the peptonized milk, in which the casein is at least partially digested, and it should be employed where there is any {220} difficulty in the way of using the ordinary milks. Either skimmed or unskimmed milk may be used for peptonizing, the latter peptonized being quite as easy of digestion as the former unpeptonized. I have found the extractum pancreatis of Fairchild Brothers & Foster most successful in the peptonizing of milk, and according to the following directions: Into a clean quart bottle put 5 grains of extractum pancreatis, 15 of bicarbonate of sodium, and a gill of cool water; shake, and add a pint of fresh cool milk. Place the bottle in a pitcher of hot water or set the bottle aside in a warm place, usually for three-quarters of an hour. When the milk has acquired a slightly bitter taste, it has been completely peptonized--that is, the casein has been completely converted into peptone. After the process is complete the milk must be immediately put on ice.

It is not always necessary to completely peptonize the milk, and if the bitter taste is unpleasant the process may be stopped short of this by putting the milk on ice, the degree of digestion depending upon the length of time the milk is kept warm.

While I am confident that the promptest and most effectual method of eliminating sugar from the urine is by a milk diet, it occasionally happens that a patient cannot or will not submit to so strict a regimen. In other instances, again, it is not necessary to resort to it, because a less restricted diet answers every purpose.

A suitable diabetic diet would also be obtained by eliminating from the bill of fare all saccharine and amylaceous and other sugar-producing substances. Such a diet is, strictly speaking, impossible. For, apart from the fact just mentioned that even fats, as well as albuminous substances to a degree, are capable of producing glycogen, the monotony of a pure meat diet soon becomes unbearable, to say nothing of other derangements it may produce. Fortunately, it is not necessary that such an exclusive diet should be maintained, for certain saccharine foods seem capable of resisting the conversion into sugar more than others. Sugar of milk, or lactin, has already been mentioned as one of these, and to it may be added the sugar of some fruits, and probably also inosit or muscle-sugar, mannite or sugar of manna, and inulin, a starchy principle abundant in Iceland moss. It is found also that there are many vegetable substances containing small quantities of sugar and sugar-producing principles which may be used with impunity in at least the milder forms of diabetes. This being the case, a bill of fare for diabetics may be constructed quite liberal enough to satisfy the palate of most reasonable persons by whom it is attainable.

FOOD AND DRINK ADMISSIBLE.--Shell-fish.--Oysters and clams, raw and cooked in any way, without the addition of flour.

Fish of all kinds, fresh or salted, including lobsters, crabs, sardines, and other fish in oil.

Meats of every variety except livers, including beef, mutton, chipped dried beef, tripe, ham, tongue, bacon, and sausages; also poultry and game of all kinds, with which, however, sweetened jellies and sauces should not be used.

Soup.--All made without flour, rice, vermicelli, or other starchy substances, or without the vegetables named below as inadmissible. Animal soups not thickened with flour, beef-tea, and broths.

Vegetables.--Cabbage, cauliflower, brussels-sprouts, broccoli, green {221} string-beans, the green ends of asparagus, spinach, dandelion, mushrooms, lettuce, endive, coldslaw, olives, cucumbers fresh or pickled, radishes, young onions, water-cresses, mustard and cress, turnip-tops, celery-tops, or any other green vegetables.

Fruits.--Cranberries, plums, cherries, gooseberries, red currants, strawberries, apples, without sugar. Or they may be stewed with the addition of bicarbonate of sodium instead of sugar. (See below.)

Bread and cakes made of gluten, bran, or almond flour, or inulin, with or without eggs and butter. Griddle-cakes, pancakes, biscuit, porridges, etc. made of these flours. Where especial stringency is required these should be altogether omitted.

Eggs in any quantity and prepared in all possible ways, without sugar or ordinary flours.

Nuts.--All except chestnuts, including almonds, walnuts, Brazil-nuts, hazel-nuts, filberts, pecan-nuts, butternuts, cocoanuts.

Condiments.--Salt, vinegar, and pepper in moderate quantities.

Jellies.--None except those unsweetened. They may be made of calf's-foot or gelatin and flavored with wine.

Drinks.--Coffee, tea, and cocoa-nibs, with milk or cream, but without sugar; also milk, cream, soda- (carbonated) water, and all mineral waters freely; acid wines, including claret, Rhine, and still Moselle wines, very dry sherry; unsweetened brandy, whiskey, and gin. No malt liquors, except those ales and beers which have been long bottled, and in which the sugar has all been converted into carbonic acid and alcohol.

Vegetables to be especially Avoided.--Potatoes, white and sweet, rice, beets, carrots, turnips, parsnips, peas, and beans; all vegetables containing starch or sugar in any quantity.

The following list, including essentially the same articles, but arranged in the shape of a true bill of fare, by Austin Flint, Jr.,[49] will be found very convenient:

BILL OF FARE FOR DIABETES.--Breakfast.--Oysters stewed, without flour; clams stewed, without flour. Beefsteak, beefsteak with fried onions, broiled chicken, mutton or lamb chops; kidneys, broiled, stewed, or devilled; tripe, pigs' feet, game, ham, bacon, devilled turkey or chicken, sausage, corned-beef hash without potato, minced beef, turkey, chicken, or game with poached eggs. All kinds of fish, fish-roe, fish-balls, without potato. Eggs cooked in any way except with flour or sugar, scrambled eggs with chipped smoked beef, picked salt codfish with eggs, omelets plain or with ham, with smoked beef, kidneys, asparagus-points, fine herbs, parsley, truffles, or mushrooms. Radishes, cucumbers, water-cresses, butter, pot-cheese. Tea or coffee, with a little cream and no sugar. (Glycerin may be used instead of sugar if desired.) Light red wine for those who are in the habit of taking wine at breakfast.

Lunch or Tea.--Oysters or clams cooked in any way except with flour; chicken, lobster, or any kind of salad except potato; fish of all kinds; chops, steaks, ham, tongue, eggs, crabs, or any kind of meat; head-cheese. Red wine, dry sherry, or Bass's ale.

{222} Dinner.--Raw oysters, raw clams.

Soups.--Consommé of beef, of veal, of chicken, or of turtle; consommé with asparagus-points; consommé with okra, ox-tail, turtle, terrapin, oyster, or clam, without flour; chowder, without potatoes, mock turtle, mullagatawny, tomato, gumbo filet.

Fish, etc.--All kinds of fish, lobsters, oysters, clams, terrapin, shrimps, crawfish, hard-shell crabs, soft-shell crabs, (No sauces containing flour.)

Relishes.--Pickles, radishes, celery, sardines, anchovies, olives.

Meats.--All kinds of meat cooked in any way except with flour; all kinds of poultry without dressings containing bread or flour; calf's head, kidneys, sweetbreads, lamb-fries, ham, tongue; all kinds of game; veal, fowl, sweetbreads, etc., with curry, but not thickened with flour. (No liver.)

Vegetables.--Truffles, lettuce, romaine, chicory, endive, cucumbers, spinach, sorrel, beet-tops, cauliflower, cabbage, brussels-sprouts, dandelions, tomatoes, radishes, oyster-plant, celery, onions, string-beans, water-cresses, asparagus, artichoke, Jerusalem artichokes, parsley, mushrooms, all kinds of herbs.

Substitutes for Sweets.--Peaches preserved in brandy without sugar; wine-jelly without sugar, gelée au kirsch without sugar, omelette au rhum without sugar; omelette à la vanille without sugar; gelée au rhum without sugar; gelée au café without sugar.

Miscellaneous.--Butter, cheese of all kinds, eggs cooked in all ways except with flour or sugar, sauces without sugar or flour. Almonds, hazel-nuts, walnuts, cocoanuts. Tea or coffee with a little cream and without sugar. (Glycerin may be used instead of sugar if desired.) Moderately palatable ice-creams and wine-jellies may be made, sweetened with pure glycerin; but although these may be quite satisfactory for a time, they soon become distasteful.

Alcoholic Beverages.--Claret, burgundy, dry sherry, Bass's ale or bitter beer. (No sweet wines.)

Prohibited.--Ordinary bread; cake, etc. made with flour or sugar; desserts made with flour or sugar; vegetables, except those mentioned above; sweet fruits.

[Footnote 49: "On the Treatment of Diabetes Mellitus," a paper read before the American Medical Association at its meeting in Washington, May, 1884, and published in the Journal of the association July 12, 1884. I have so far modified the bill of fare as to permit the use of milk, which Flint excludes.]

One of the foods the omission of which is most illy borne by the diabetic, however great his previous indifference to it, is wheaten bread, while the substitutes which have been at different times suggested for it very imperfectly supply its place. Perhaps the best known of these is the bread made of gluten flour. It was suggested by Bouchardat in 1841, and is made by washing the ordinary wheat flour to free it from starch.[50]

[Footnote 50: The Health Food Company, of 74 Fourth Avenue, N.Y., prepare a gluten flour by first removing the five bran-coats, pulverizing the cleaned berry by the cold-blast process, stirring the powder into iced water, and precipitating the gluten, cellulose, and mineral matters, siphoning off the water holding in suspension the starch, and drying out the precipitate. In this manner the salts of the wheat are retained. A purified gluten made by the Health Food Company is deprived of the cellulose walls of the cells in which the gluten granules are held. Directions for making gluten bread and cakes of various kinds are furnished by the company on application.]

Gluten flour, however prepared, contains some starch, as indeed it must if bread is to be made out of it; and I confess to having been a good deal disappointed in its use. I have known the sugar absent in a {223} selected diet to return when gluten bread was permitted, and again disappear on its withdrawal. Of course gluten flour contains less starch than the ordinary wheat flour, and there may be cases where the starch in the former can be assimilated when the quantity in the latter cannot be. The gluten may be made into porridge.[51]

[Footnote 51: Gluten porridge is made by stirring the gluten into boiling water until thick enough, and then keeping up the boiling process for fifteen minutes. A little salt and butter are added at the close to improve the flavor, and it may be eaten with milk or cream.]

A method of getting rid of the starch and sugar in bread, suggested by Liebig and tried by Vogel, consists in converting the starch into sugar by the action of diastase and dissolving out the sugar thus produced. This is accomplished by treating thin slices of bread with an infusion of malt. The bread is then washed, dried, and slightly toasted.

Another substitute for wheaten flour is the bran flour whence the starch is removed by washing.[52] The bran itself, according to Parkes,[53] sometimes contains as much as 15 per cent. of nitrogenous matter, 3.5 per cent. of fats, and 5.7 per cent. of salts. It is therefore not wholly innutritious, although the salts are washed out in removing the starch. It is considered especially useful when there is constipation, the slightly irritant properties of the bran aiding in maintaining a proper peristalsis and action of the bowels. These irritant properties are, however, inversely as the degree of comminution. The bran flour may be made with milk and eggs into a variety of cakes, of which the best known are those made according to Camplin's directions.[54]

[Footnote 52: A very carefully prepared bran flour, as well as a wheat-gluten flour, is prepared by John W. Sheddon, pharmacist, corner of Broadway and Thirty-fourth street, New York City.]

[Footnote 53: Practical Hygiene, 5th ed., Philadelphia, 1878, p. 222.]

[Footnote 54: The following are Camplin's directions for making biscuit of bran flour: To one quarter of a pound of flour add three or four fresh eggs, one and a half ounces of butter, and half a pint of milk; mix the eggs with a little of the milk, and warm the butter with the other portion; then stir the whole together well; add a little nutmeg or ginger or other agreeable flavoring, and bake in small forms or patterns. The cake, when baked, should be about the thickness of an ordinary captain's biscuit. The pans must be well buttered. Bake in rather a quick oven for half an hour. These cakes or biscuits may be eaten by the diabetic with meat or cheese for breakfast, dinner, or supper; at tea they require rather a free allowance of butter, or they may be eaten with curd or any soft cheese.]

Where extreme restriction of diet is not required the ordinary bran bread of the bakers may be used. The unbolted flour of which this is made of course contains the starchy principles, but in consequence of the retention of the bran the proportion of starch is less. The cold-blast flour of the Health Food Company is said to contain the nutritious, but not the innutritious, parts of the bran.[55]

[Footnote 55: It is made by pulverizing the carefully cleaned wheat by a compressed, cold air blast, which strikes the wheat and dashes it to atoms.]

The almond food suggested by Pavy is another substitute for bread. The almond is composed of 54 per cent. of oil, 24 per cent. of nitrogenized matter known as emulsin, 6 per cent. of sugar, and 3 per cent. of gum, but no starch enters into its composition. Theoretically, therefore, the food should be everything that can be desired if the gum and sugar can be removed. The latter is done by treating the powdered almonds with boiling water slightly acidulated with tartaric acid, or soaking the almonds in a boiling acidulated liquid which may form a part of the process for blanching. The boiling and acid are necessary to precipitate {224} the emulsin, which would otherwise emulsify the oil of the almond. Pavy speaks well of biscuit made of almond flour and eggs, which he says go very well with a little sherry or other wine, although he admits they are found too rich by some for ordinary consumption. One person only under my observation has used the almond food, and found it unpalatable.

Seegen recommends an almond food made as follows: Beat a quarter of a pound of blanched sweet almonds in a stone mortar for about three-quarters of an hour, making the flour as fine as possible; put the flour thus obtained into a linen bag, which is then immersed for an hour and a quarter in boiling water acidulated with a few drops of vinegar. The mass is thoroughly mixed with three ounces of butter and two eggs; the yolks of three eggs and a little salt are added, and the whole is to be stirred briskly for a long time. A fine froth made by beating the white of the three eggs is added. The whole paste is now put into a form smeared with melted butter and baked by a gentle fire.

Biscuits made of inulin, the starchy principle largely contained in Iceland moss, were suggested by Kuelz. Although a starch, it is one of the assimilable ones alluded to, of which small quantities at least may be taken as food without appearing in the urine as sugar. The biscuits are made with the addition of milk, eggs, and salt, and are inexpensive.

To some persons sugar is almost as imperative a necessity as bread, although to many it is not a very great sacrifice to omit it from ordinary cooking, if not from tea and coffee. For the latter it is just as well to dispense with sugar altogether. But where patients feel that they must have some substitute for sugar, glycerin has been suggested for this purpose, at least for sweetening tea and coffee. But Pavy has noted[56] that under the use of glycerin the urine increased from three and three and three-fourth pints to between five and six pints, and the sugar from 1100 grains to 3000 grains per diem, in the course of three days. Its withdrawal was followed by a prompt fall in both the urine and sugar, a return to it by a second increase, and subsequent withdrawal by another decline. Along with the increase of urine and sugar came also more thirst and discomfort. An examination of the chemical composition of glycerin would seem to confirm these results of experience. Glycerin is represented by C_{3}H_{8}O_{3}, sugar by C_{6}H_{12}O_{3}, and glycogen by C_{6}H_{10}O_{5}; whence it is evident that a conversion of glycerin into sugar may take place in the liver. These facts seem to show conclusively that glycerin is no suitable substitute for sugar. I therefore do not use it.

[Footnote 56: On Diabetes, London, 1869, p. 259.]

From what has been said it may be inferred that sugar of milk, mannite, and lævulose, or fruit-sugar, are admissible where sugar is demanded. They may be tried, but the urine should be carefully examined under their use, and if glycosuria occur or be increased they should be promptly omitted.

Almost every purpose of sugar in the cooking of acid vegetables is served by bicarbonate of sodium or potassium. As much bicarbonate of potassium to the pound as will lie upon a quarter of a dollar will neutralize the acidity of most fruits which require a large amount of sugar to mask this property. In this manner cranberries, plums, cherries, gooseberries, red currants, strawberries, apples, peaches, and indeed {225} all fruits to which sugar is usually added in the cooking, become available to the diabetic.

In the matter of drinks, where the patient is not on a skim-milk diet, which usually affords as much liquid as is required by the economy, little restraint need be placed upon the consumption of water, which is demanded to replace that secreted with the sugar. Instead of water, Apollinaris water, Vichy, or the ordinary carbonated water may be used if preferred, and to many they are much more refreshing by reason of the carbonic acid they hold in suspension. Apollinaris water is particularly so, and one of my patients, who recovered completely under a suitable selected diet with which this mineral water was permitted, insists that it was that which cured her.

Where a simple selected diet is adopted, tea and coffee without sugar are usually permitted. The propriety of the substitutes for sugar already referred to must be determined by circumstances.

Of distilled and fermented liquors, moderate quantities of whiskey and brandy, dry sherry and madeira, the acid German and French wines--in fact, any non-saccharine wines--may be permitted. A medical friend who reports himself about cured of diabetes writes me that he has consumed eighty gallons of Rhine wine since he began to adhere closely to a diabetic diet. On the other hand, the free use of the stronger alcoholic drinks has been charged with causing diabetes, and I have known such use to produce a recurrence of sugar. No malt liquors, except those in which the sugar has been completely converted into carbonic acid and alcohol, should be used. Bass's ale may be allowed where no especial stringency is required.

HYGIENIC TREATMENT.--The patient should be surrounded by the most favorable hygienic influences. He should sleep in well-ventilated rooms; pass much time in the open air; bathe regularly, but not in water that is very cold, and especially the body should not be long submerged in cold water, as the liver must share the general internal hyperæmia incident to prolonged cooling of the skin, and increased glycosuria may result. I have known sugar to reappear after a prolonged drenching of the skin of patients overtaken by a rainstorm. Perhaps the most suitable time for the hot or tepid bath is on retiring in winter, but in summer it may be taken on rising. Thorough friction of the entire body should be practised after the bath or independently of it. An ounce or two of sodium carbonate may be added to it with advantage, as it softens the skin and facilitates the removal of the effete epithelium. The bowels should be kept regularly open, as the effect of their confinement is to produce torpor and congestion of the liver.

Certain natural mineral waters have always enjoyed a reputation for the cure of diabetes, and notably those of Vichy and Carlsbad. The former is an alkaline water with a slight laxative tendency, and the latter a decided aperient alkaline-saline water; and it is not unlikely that they owe a part of their good effects to an action upon the liver and upper bowel. This seems the more likely because Carlsbad, which enjoys the highest reputation, contains a far larger proportion of chlorides and sulphates, which are purgative. Vichy water contains 35 grains of carbonates to the pint, and Carlsbad 11, but the latter contains twice the proportion of chlorides, or 8 grains to the pint, and ten times as much sodium {226} sulphate, or 19 grains to the pint. They may be used as adjuvants to the treatment, a pint of Vichy or half as much Carlsbad in the morning. Being imported waters, they are comparatively expensive, and I know of no American waters which closely approach them in composition.

Of American waters, the Saratoga Vichy contains twice as much chlorides as the Carlsbad, 17.7 grains to the pint, but no sulphates. It contains about the same amount of carbonates as Vichy. It is therefore a saline-alkaline water, and may be expected to serve the purposes of Vichy and some of those of Carlsbad, for which it may be substituted. Most of the American mineral waters vaunted as useful in diabetes will be found, on comparison with these waters, to be chemically indifferent, and therefore about as useful as so much ordinary spring-water. Of the Crab Orchard Springs in Kentucky, the Sowder's spring contains 25 grains of sulphate of sodium and magnesium and 7 grains of sodium chloride to the pint, therefore about the same proportion of the two substances combined as Carlsbad; yet I am not aware that these waters have any reputation in diabetes. The waters of Bedford Springs, Pennsylvania, also approximate them in the proportion of sulphates of sodium and magnesium.

Other Saratoga waters have an undoubted action on the liver through their chlorides, and may be used in lieu of the European waters above referred to, and of the Saratoga Vichy, when these cannot be obtained; such are the Geyser spring, which contains 70 grains of chlorides to the pint, and the Hathorn, containing 63 grains.

MEDICINAL TREATMENT.--While the dietetic treatment, and especially the skim-milk treatment, of diabetes mellitus is much to be preferred for its results over an exclusively medicinal treatment, and is of itself sufficient to control, if not to cure, a large number of cases, yet instances arise in which it is insufficient to complete the removal of sugar from the urine, and there are others in which it is impossible for various causes to carry out such treatment.

In my book on Bright's Disease and Diabetes, published three years ago, I gave the preference of drugs to ergot; but since then extended opportunities have convinced me that codeia is a far more efficient remedy. Repeated comparative trials of this drug in the wards of the Philadelphia Hospital and elsewhere have satisfied me of this. The trials have been made while the patients were upon a mixed diet, which I hold to be the only fair way of arriving at a knowledge of the true value of a drug in the disease. Codeia was first suggested by Pavy in lieu of opium and morphia, which had long been used, his reason being that it did not produce the same narcotic effect. Favorable reports upon its use have been made by Foster, Image, Brunton, R. Shingleton Smith, Cavafy, Austin Flint, Sr., Harvey L. Byrd, and others. It may be given in pill or solution. One should begin with ¼ of a grain three times a day, increasing ¼ of a grain daily until the sugar disappears or the remedy ceases to have any effect, or until drowsiness is produced. Thus gradually increasing, I have reached as high as 47 grains in a day. Cavafy has given 15 grains three times daily.

Opium--which is said to have been used by Aetius for this disease--or morphia might be used if codeia cannot be obtained, but they are less efficient, more dangerous, and more apt to produce the troublesome {227} symptom of constipation. MacGregor[57] gave in one case 60 grains of opium, and in another 90 grains, in the twenty-four hours.

[Footnote 57: London Medical Gazette, 1837.]

While I have seen the most striking results upon the quantity both of sugar and urine during the administration of codeia, and at the same time have noted a gain in flesh and strength, I cannot say that I have ever seen a case totally recover under its use. Such cases are, however, reported by others. I have always used it in the very worst cases, where dietetic measures had also failed to remove the sugar. As to the mode of action of codeine, we can only speculate. It may be said that it quiets the irritation of the vaso-motor centre, whence result the glycosuria and other symptoms of diabetes.

Next to codeine in efficiency, of drugs, is ergot. The favorable results of its use are more easily explained by its physiological action--contraction upon the muscular walls of blood-vessels--than those of codeine, but it is not so efficient a remedy. It may be used by beginning with half a drachm, and increasing to a drachm, four times a day. Larger doses than this, as much as half an ounce four times a day, have been given, but the stomach rarely permits their continuation for any length of time.

Bromide of potassium, an old remedy for diabetes, has recently been revived and much lauded by the French physicians, but I have never found any results from its use. I can understand, however, how in certain cases of nervous origin it may be useful.

Comparatively recently, Clemens of Frankfort-on-the-Main has recommended the use of what he terms brom-arsen or bromide of arsenic. The dose is one-forty-eighth of a grain three times a day, gradually increased by this same amount until one-sixth or one-fifth of a grain is given daily.[58] Clemens, however, unites with its use a dietetic treatment. I have used it in connection with an unselected diet, and have not found the results claimed by Clemens. It is, however, both tonic and sedative, and as such is to be recommended in conjunction with other measures.

[Footnote 58: The late Mr. R. F. Fairthorne, with Mr. James T. Shinn, apothecary, cor. Broad and Spruce streets, Philadelphia, prepared for me a solution of bromide of arsenic in the following manner: 77 grains of metallic arsenic in powder are added in small portions to 240 grains of bromine, the latter being placed in a long test-tube immersed in ice-water to control the otherwise violent reaction. One hundred grains of the tribromide thus obtained are dissolved in sufficient distilled water to make ten fluidounces. One minim will then contain one-forty-eighth of a grain.]

Arsenic itself has some reputation in the treatment of diabetes, based upon the observation of Salkowsky that glycogen diminishes in the livers of animals poisoned with arsenic. It is at least a good adjuvant tonic. Leube gave it in diabetes in doses of one-third of a grain three times a day.

Strychnia is also very useful as a tonic, and may be used either alone or in the shape of the sulphate, or combined with arsenic and iron, or it may be given, perhaps preferably, in solution in combination with an acid. Given in combination with phosphoric acid, I believe it the most valuable tonic available in this disease.

To supply the phosphates, in which gluten bread is deficient, as well as for their tonic effect, the various preparations of phosphates are useful. {228} The well-known compound syrup of the phosphates, or Parrish's chemical food, may be considered a type of these preparations. Every fluidrachm, which is a dose, contains 2½ grains of calcium phosphate, 1 grain of ferric phosphate, with fractions of a grain of sodium and potassium phosphate with free phosphoric acid. Similar is the solution of phosphates and phosphoric acid[59] known in this country as solution of phosphoric acid with iron, or the latter may be omitted.

[Footnote 59: Rx. Calcii phosphat. gr. iij; Magnesii " gr. ss; Potassii " gr. iv; Ferri " gr. ss; Ac. phosphoric minim vj; Aquæ q. s. ad. fluidrachm i, which is a dose.]

Iodide of potassium has been used in some cases with satisfactory results, and may be expected to be useful where syphilitic disease of the nervous system is suspected.

Seegen has seen sugar disappear from the urine under a dosage of 20 to 30 drops of tincture of iodine daily, but the sugar reappeared after the remedy was discontinued.

Lactic acid was recommended by Cantani on theoretical grounds as a substitute for sugar. He supposes that in health the sugar ingested is converted by the liver into lactic acid, and he would furnish the latter already formed, and thus spare the liver this function. Senator also favors the use of this acid for a similar purpose, but reasons that in health sugar is converted into lactic acid in the small intestine, while in diabetes this conversion is interfered with. Hence, too, it should be given fully formed. Patients under its use are said to gain in weight and to become stronger, while it is not claimed that it alone diminishes the glycosuria; this must be brought about by a selected diet. The lactic acid is simply an important force-producer not otherwise obtainable, because sugar fails to undergo its usual conversion. Cantani recommends that from 75 to 150 grains of the acid should be taken daily in from 8 to 10 fluidounces of water. Diarrhoea and pains in the joints are said to follow the use of large quantities of the drug, but these again disappear on its omission. My experience is limited to a single case, which recovered while taking 30 drops three times a day in conjunction with Carlsbad water and a pill of iron, quinia, and arsenic.

Senator suggested that the fatty acids--oleic, palmitic, stearic, and butyric--be used on the same principle that lactic acid is given, that their force-producing power may be availed of. To this end he prescribed, with partially satisfactory results, soap in pills containing 2-1/3 grains each, of which four were taken daily.

Cod-liver oil is especially suitable as a food where debility is to be combated. Even those who claim that fats are convertible into sugar in the liver admit that it is only in the most advanced stages of diabetes that such conversion takes place. Cod-liver oil, therefore, in common with other fats, may form part of a diabetic diet, and is especially indicated where phthisis is present, as it so often is, in the latter stages of the disease, or indeed whenever a good tonic is indicated.

In 1882, Moleschott[60] suggested the use of iodoform in diabetes. He reported the effect of its use in five cases, giving .1 to .3 grm. (1.5 to {229} 4.5 grs.) in pill with extract of lactucarium and cumarin, the purpose of the latter being to disguise the odor. His formula was as follows: Iodoform, 1 gram (15 grs.); ext. lactuc. sat., .1 gram (15 grs.); cumarin, .1 gram (1.5 grs.), to be made into twenty pills. In one case the sugar disappeared in twelve days; in the second, at the end of six months; in the third case it had diminished from 14.4 to 1.6 grams in three months; in the fourth, from 28 grams to 1.6 in four months; and in the fifth case, from 9.2 to 6.1 grams.

[Footnote 60: Wiener Med. Wochenschr., Nos. 17, 18, 19.]

The use of the remedy in Moleschott's hands produced no unpleasant results, but Drasch,[61] who used the same treatment after Moleschott's method in three cases, with the effect of diminishing the thirst, the quantity of urine, and the proportion of sugar, found excessive itching of the skin, diminished appetite, and diarrhoea to result in such degree as to demand its disuse in the majority of cases. Iodoform has been used by the Italian physicians De Renzi,[62] Bozzolo,[63] and Silvestrini,[64] and by Sara E. Post[65] of New York, with varying but generally favorable results, except in Silvestrini's case. These results included diminution in thirst, quantity of sugar and urea, with increase in weight. The drug deserves a trial in doses of from 1 to 2 grams (15 to 30 grains) a day, but due regard should be had to possible toxic effects; and to this end the administration should be interrupted at the end of one or two weeks, and the interruption continued for a like period. It may be given in pill or in capsule, and in divided doses or in a single dose at bedtime. The latter course is recommended by Post, and is said to avoid eructations and anorexia. Theories of its action based upon experimental use of poisonous doses ascribe its effect to a primary stimulating and ultimately fatally degenerative effect upon the protoplasm of cells, and especially those of the liver and nervous system.

[Footnote 61: Wiener Med. Presse, 1882, xxiii. 1487.]

[Footnote 62: "Tre Storie di Diabete." Gior. internaz. d. sc. med., Nap., 1882, N. S. iv. 913-917.]

[Footnote 63: "Sur l'action du iodoforme dans la diabète sucre," Arch. ital. de biol., Turin, Feb., 1883, iii. 317-321.]

[Footnote 64: "Iodoforme dans le diabète," La France Méd., October, 1883, ii. 567.]

[Footnote 65: Archives of Medicine, April, 1884, p. 116.]

Transfusion of blood has been recommended by Dieulafoy,[66] and is approved of by Ralfe,[67] especially to combat the symptoms of acetonæmia, which, if due to a toxic agent, as seems most likely, should be met by altering the percentage composition of the blood with relation to the toxic agent.

[Footnote 66: "Étude sur la Transfusion du Sang dans le diabète sucre," Bullétin et Mém. Soc. Méd. de Hôp. de Paris, 1884, 4, S. 1, 38, 41.]

[Footnote 67: "Discussion before the Path. Soc. of London," Lancet, Apr. 7, 1883, p. 592.]

Diabetic neuralgia yields generally to the treatment of the disease in general correspondingly to the reduction in the quantity of sugar, and at times to salicylate of sodium, while it does not respond to morphia or other remedies for ordinary neuralgia.

The alkalies, which attained some reputation after Mialhe claimed for them the power of destroying sugar in the blood and of neutralizing the fatty acids which were thought to accumulate there in consequence of the deficient action of the skin, are not often used at the present day. Potassium carbonate was the favorite preparation, and in the hands of Pavy its use seems to have been followed by good results. He gave it in 10, 15, and 20 grain doses in combination with aromatic spirit of {230} ammonia. Sodium bicarbonate was less satisfactory, as were also potassium acetate, potassium citrate, and Rochelle salts. These were given in doses of from four drachms to an ounce daily. In Germany, too, the alkaline treatment has been used to some extent.

As is the case with so many diseases which are incurable by any special treatment, a large number of remedies have at different times been suggested for diabetes, mostly on a foundation which does not admit of close analysis. One of these was the nitrate of uranium, suggested by Dale of Lemont, Pennsylvania, who gave it in doses of 1 grain three times daily, increased to 3 if necessary, in pill, powder, or solution, by aid of a few drops of nitric acid. He appears, however, to have used it in connection with a selected diet. I have tried it both with and without a selected diet; in the latter case there was no effect, and in the former there was none which the diet alone would not have produced.

Sodium phosphate, salicylic acid, salicylate of sodium, have all been used, it is claimed, with good results, and the late Dr. Dougherty of Newark, New Jersey, used with apparent advantage a mixture into which all of these, together with sodium carbonate, entered, made up with glycerin, tincture of cardamom, and water, the doses being 2½, 2½, 4½, and 8½ grains respectively. Moleschott has also obtained good results with salicylic acid.

{231}

SCROFULA.

BY JOHN S. LYNCH, M.D.

SYNONYMS.--Scrophula, Scrofulosis, Morbus scrophulosus, Struma, King's evil, The evil, Quince, Cruels and Crewels (Scotice).

DEFINITION.--A morbid condition of the system manifested by a peculiar liability to certain forms of nutritive disorders of the skin, mucous membranes, joints, bones, organs of special sense, and especially the lymphatic glands.

There is probably no disease of which it is more difficult to give an exact and satisfactory definition than scrofula. The general tendency of medical opinion within the last few decades has been to narrow the significance of the term, and even to restrict it to those slow and indolent inflammations and over-growths of lymphatic tissue which end in caseation and finally imperfect suppuration. Formerly almost every deviation from healthy functional activity in the young, as well as every disorder of nutrition which could not be assigned to any definite cause, was called struma; and thus, as Heule well remarks,[1] "Scrofula became the receptacle into which one vaguely casts all the ailments which afflict children under fourteen years, and of which we do not know the cause."

[Footnote 1: Handbuch der Rationellen Pathologie.]

Before hereditary syphilis was understood all its manifestations were classed as scrofulous, and at least one eminent authority in the United States[2] has expressed the opinion that scrofula is only a manifestation of the syphilitic poison in the second or third generation. Rickets, chronic hydrocephalus, favus, lice, and worms (Lugol), diabetes (Carmichael), and even scirrhus and cancer (R. Hamilton), have all been classed as scrofulous diseases. Then there is a large class of unhealthy persons whose morbid state can be no more definitely expressed than by saying that they are "delicate" or of "feeble health" or of "frail constitution," and by some all these are included under the term scrofulous. But as knowledge advances, and pathological knowledge as well as diagnostic acumen becomes larger and keener, many of these affections and morbid conditions can be eliminated from scrofula and assigned their true pathological and nosological position.

[Footnote 2: S. D. Gross, Transactions American Medical Association, 1878.]

To many who have been educated in the more modern schools of medical thought, therefore, our definition will appear much too broad, while to others it may appear too narrow.

Scrofula is essentially and purely a diathetic, not a cachectic, disease. {232} It is true that what may be called the manifestations or lesions of the disease are often excited by some preceding dyscrasia, and also that the long persistence of these lesions may excite a cachectic condition which we might call the scrofulous cachexia; still, as many children suffer from the lesions of scrofula who have never exhibited any evidence of a precedent dyscrasia, but on the contrary appear to be in perfect health, and many others, on the other hand, show unmistakable evidence of ill-health and are decidedly dyscrasic, yet are never attacked by scrofula, it is believed that every subject of scrofula becomes so not because of any pre-existing dyscrasia or cachexia, but because of some peculiar condition of the system--innate or acquired--which constitutes a diathesis.

"The hypothesis," says Niemeyer,[3] "that scrofula depends upon a faulty composition of the blood (dyscrasia), and that the lesions found in scrofulous persons were due to a deposit in the tissues of a matter circulated by the blood and called a scrofulous material, is almost universally abandoned."

[Footnote 3: Textbook of Practical Medicine, vol. ii.]

But while insisting upon the peculiar and, so to say, specific origin of the disease in some special condition of the system, without which it will never exist, it is admitted that the lesions of scrofula do not differ essentially from other similar lesions of the same tissues of a non-scrofulous origin. They are mostly of an inflammatory nature, and are only to be distinguished by the often trivial character of their exciting causes--often, indeed, by the total absence of any known exciting cause--and by their tediousness and intractability.

ETIOLOGY.--We believe, as already stated above, that the essential cause of scrofula is some peculiarity in the constitution of the tissues of the scrofulous subject; and we think it highly probable that H. F. Formad of Philadelphia has pointed out what constitutes this peculiarity. He declares--and the correctness of his observation has been abundantly verified--that microscopic examination of the tissues of certain animals characterized by their extreme aptness to be affected by scrofula and tuberculosis, as well as of children known to have been scrofulous or tuberculous, discloses the fact that the lymph-spaces in these subjects are always more numerous, larger, and more crowded with cells than in non-scrofulous subjects. The tissues of the scrofulous are therefore coarser, less compact and solid, and there is a greater tendency to undue cell-growth, than in the non-scrofulous. And these are precisely the characteristics which they present clinically, and such as we might have, a priori, expected to find.

This peculiarity of anatomical structure is in a large number of cases undoubtedly inherited from the parents, but while heredity plays, as is well known, an important part in the etiology of struma, it is not the essential factor. Bad hygienic surroundings, overcrowding, and consequent want of fresh air, improper food, consisting of a too great proportion of starch, during the early months or years of life, will cause the growing tissues to assume the peculiar anatomical arrangement alluded to above. "A coarse diet, containing but little nourishment in comparison with its bulk, is especially held in evil repute. The earlier this injudicious feeding of an infant commences, so much the greater danger that it will become scrofulous; hence the children fed on pap furnish a very important contingent to the army of scrofulous persons."[4] The {233} well-known fact that few children at the breast suffer from scrofulous lesions, but that a large number do so within the first two or three years after weaning, certainly tells in favor of the belief that too much starch and an insufficiency of animal food favor, if they do not actually produce, that faulty nutrition and construction of tissues which we believe lies at the foundation of the scrofulous diathesis. Independent, however, of improper food and the other predisposing causes mentioned, it is quite probable that faulty nutrition caused by accidental disease of the digestive or assimilative organs during infancy may create a predisposition. How else can we account for those not very rare cases in which from parents perfectly free from any scrofulous taint a large family of children may be reared, of which only one will suffer from any scrofulous lesions? Two such instances have been brought to my notice, and as the children in these cases lived upon a farm on the water-side, and enjoyed an abundance of pure air and salt-water bathing, and were certainly not stinted in food of proper quality, it is difficult to account for the acquired diathesis except upon the hypothesis above. Among the general predisposing causes of scrofula in addition to the special ones I have mentioned may be added--

[Footnote 4: Niemeyer, loc. cit.]

1. Locality and Climate.--It has long been believed that scrofula is more common in the temperate zone than in the extreme north or in the tropics. While this is probably true, it must be stated that a sufficient amount of reliable statistics bearing upon this point have not yet been collected to prove the fact beyond cavil. That we should find that the disease prevails more extensively in cold and damp situations than in warmer and drier ones is to be expected, since the former conditions involve a greater confinement within dwellings, and consequently a diminished supply of fresh air, which, as we have seen, constitutes one of the predisposing causes of scrofula. Moreover, it is in these situations we would encounter a greater number of catarrhs, which, as we shall see, are known to be among the most active of the exciting causes of the glandular affections of scrofula.

2. Season.--For the same reason we find that a large number of cases of scrofula make their appearance in the early spring months, the results of catarrh contracted during the previous winter or of the sudden changes of temperature which accompany the transition of winter to summer.

3. Age.--Scrofula is essentially a disease of early life, but not exclusively so. As the diathesis can only be acquired directly from the parent, or fortuitously by malnutrition during the period of active growth, it follows that it becomes established, if at all, before the age of twenty years. And as the predisposition seems to be quite strong in most cases, and as the exciting causes are more apt to be applied during the earlier years of life, it is not surprising that a very large majority of the cases occur between the ages of three and fifteen years. A few, however, escape during childhood, and only suffer from it between twenty and thirty, while a small number only develop the disease in old age. Rindfleisch mentions the period between twenty and thirty as a common one for the development of hereditary scrofula; and senile scrofula was first pointed out by Sir James Paget.[5] In all these cases of deferred {234} manifestation of the scrofulous diathesis--and they are not very numerous--it is to be presumed that they have escaped the most active of the exciting causes of the disease. Indeed, it is natural that a person having inherited the predisposition should be more sedulously guarded--at first by his parents, and afterward by himself--against the exciting causes of scrofula during infancy and adolescence.

[Footnote 5: Clinical Lectures and Essays, London, 1875.]

4. Sex.--There is no reason to believe that sex plays any part in the predisposition to this disease. Both sexes seem to be affected in about equal proportions, but from the statistics bearing upon this point it does seem to have some influence in determining the variety of its lesions. Thus, females seem to be more frequently affected with glandular disease, while males suffer from diseases of the joints in the form of coxalgia, white swelling of the knee, and Pott's disease.

5. Condition in Life: Social Position.--If what we have said about the predisposing influence of improper or insufficient food, overcrowding, etc. be true, it will naturally be inferred that a large proportion of the cases of scrofula will be found in the lower strata of society; and this is true. Especially in cities, where the disease prevails most extensively, we always find that the denizens of narrow streets, lanes, and alleys furnish the largest contingent to the deaths as well as the deformities from scrofula. It is here that the poor congregate to avail themselves of the cheaper rents, and here will be found combined all those predisposing causes which may be briefly summed up in one word--poverty. It is true that cases of scrofula are quite numerous in the country, and in a note to Sir Thomas Watson's Practice of Physic (1851) D. Francis Condie quotes from a work on The Nature and Causes of Scrofula, by Phillips, statistics which showed a greater preponderance of deaths from scrofula in a given number of the rural population than a nearly equal urban one. But at the time these statistics were gathered in England (and perhaps now) it is probable that there was a comparatively greater number of abjectly poor people among the rural population than in London, where was congregated such a large number of small tradesmen, artisans, and laborers, who, though not well-to-do, were better paid, and consequently lived better, than the agricultural laborers. Of course, a certain number of cases of scrofula are found in the United States, and perhaps in all other countries, among the children of the wealthy. These, however, are almost invariably caused either by direct transmission from parents or by some accidental injury to the digestive and assimilative organs in early childhood, as we have already pointed out. When it is remembered that in the constantly changing fortunes which are so frequently witnessed in this age of excessive activities, and that in the grand opportunities for obtaining wealth furnished by the liberal institutions and rapidly-growing industries of the United States the descendant of the pauper of the last generation may be the millionaire of the present, it is not surprising that so many who are now wealthy may possess the strumous diathesis as an inheritance from their parents or grandparents, and which they in turn transmit to their offspring.

6. Consanguineous Marriage.--It has long been a popular belief that the offspring of parents closely related by blood are more apt to be scrofulous than when no such relation has existed. Indeed, not only scrofula, but numerous other diseases, deformities, and imperfections have been {235} ascribed to such unions. Idiocy or feeble-mindedness has also been especially accredited to the production of such marriages. But a thorough investigation of this point in England some years ago demonstrated positively that no more idiotic, feeble-minded, or insane children are born of such marriages than of an equal number of marriages contracted between persons not related by blood to each other. There is, however, this amount of truth in the popular belief: if persons closely related to each other possess the scrofulous diathesis, there will be a greater probability--almost certainty--that the diathesis will be transmitted to their offspring. If one parent only is tainted with scrofula, and the other is entirely free from it, there is a possibility--even a probability--that some or all of the children may escape.

7. Complexion and Temperament.--It has been stated by some observers that scrofula occurred principally in the fair-haired, and with equal positiveness by others that it was in the dark-haired that the disease found the most of its victims. Such statistics as have been furnished, however, upon this subject seem to show that there is no connection whatever between scrofula and complexion. It will generally be found that whenever in any country or locality more cases of scrofula occur in persons of one or the other of the complexions, it is only because that particular complexion is the predominant type among the inhabitants of that locality.

8. Race and Nationality.--While it would seem that no race or nation is entirely free from struma, yet there are certainly in the United States two peoples who furnish an enormously disproportionate number of scrofulous cases: these are the Irish and Jews. Among the first of these both scrofula and tuberculosis abound with exceeding frequency, while among the latter it is scrofula alone which seems to predominate. The last, however, are not exempt from tuberculosis, but only exhibit about an equal predisposition to it with their fellow-citizens. It is not difficult to explain the special predisposition of these peoples to scrofula when their past history is taken into account in connection with what has been said about the bad influence of food and surroundings in producing the scrofulous diathesis. The principal food of the Irish peasantry--oppressed and ground into poverty by their Anglo-Saxon conquerors for hundreds of years--have been bread and potatoes, often potatoes alone. It cannot be surprising, therefore, that Irish children fed upon this diet and reared in ill-ventilated hovels should develop the scrofulous diathesis in legions. The Jews, too, oppressed by all nations through ages, have been during many generations reared in poverty and squalor. Even those of them who in not very remote times had acquired by thrift the means of securing both the comforts and luxuries of life dared not live according to their means, lest a show of wealth should attract the unpleasant, often fatal, attention of their rapacious and unscrupulous Christian or Mohammedan neighbors. This condition, this mode of life, has existed among them for many hundreds of years, and has so intensified the strumous diathesis among them that almost the whole race may be said to be patently or latently scrofulous. The negro or African race, however, as observed by the writer in the Southern States of the American Union, do not seem to have developed any special predisposition to struma, notwithstanding their servile condition. This, at first sight, would seem to {236} be contrary to our expectation based on what has been said about Jews and Irishmen. But as my remark has been predicated only on observation of the African in the Southern States, where the climate is not favorable for the development of scrofula, the fact is not so surprising. Besides, the food of these people consisted largely of bacon or pork, fish, milk, and the succulent fruits and vegetables, with a moderate quantity of corn bread, and very rarely potatoes. As the rude cabins in which they dwelt were usually constructed of unhewn logs and covered with rough boards, and cost almost nothing except labor, overcrowding was unknown and ventilation always perfect. The waiter practised medicine fourteen years in Wilcox county (S. W.), Alabama, containing a population in 1870 of 28,377, of whom 21,610 were colored, and during this time saw only two cases of genuine scrofula and one of tuberculosis among the colored population.

Pork as an article of food has often been accused of producing a tendency to scrofula, but evidently with great injustice, for we have seen that the Jews, who never eat it, are almost universally scrofulous, while the Southern negroes, whose staple animal food it was, were conspicuously free from it.

9. Acquired Scrofula.--Although in perhaps a majority of all scrofulous cases the diathesis has been inherited from the parents, the fact cannot be too strongly emphasized that in a large number of cases the disease may be developed de novo, independent of such heredity. To scrofula developed from the influence of bad ventilation and overcrowding, absence of sunlight, insufficient, bad, or unsuitable food, cold and damp, imperfect clothing--in short, all those conditions associated with poverty, squalor, and ignorance--Grancher has well applied the term la scrofula a miseria. And it is only by a clear comprehension that scrofula may be, and often is, developed under these conditions that the medical profession in general, and municipal health authorities in particular, may be induced to teach and enforce upon the poor both the knowledge and the practice which may prevent it. Even in the open country, where there is at least no lack of pure air and light, the lesson can be enforced with equal profit; for the children of the farm-laborer are likely to be imperfectly and improperly fed, and lodged in apartments at night that in the matter of foul air and filth could not be well surpassed in the purlieus of the dirtiest and most overcrowded city.

EXCITING CAUSES.--The actual exciting causes of scrofula when the diathesis already exists are too numerous to be mentioned in detail. Indeed, almost any trivial injury or inflammation, any disease which has produced a temporary cachexia, may rouse into activity the perhaps hitherto latent tendency. How often do we see a slight blow upon the knee-joint produce a white swelling which lames for life the heretofore healthy and active boy or girl! A fall upon the hip which was almost unnoticed at the time excites a coxalgia which either destroys life or renders the child for life a cripple; or a slight jar of the spine induces a disease of the vertebræ which, if recovered from at all, produces a terrible deformity. A slight eczema of the face or scalp or a catarrh of the mouth or throat will excite that slow and generally painless enlargement and induration of a neighboring lymphatic gland which always ends in its caseation and final destruction by suppuration. A slight injury to the {237} periosteum may excite a destructive caries or necrosis of the underlying bone, and a temporary catarrh of the intestines a tabes mesenterica with all its fatal consequences.

Speaking generally, it may be said that anything that produces a local disorder of nutrition or impairs the health generally of a person predisposed to scrofula is sufficient to bring about some manifestations of the disease. They are especially apt to follow the eruptive fevers. Measles and scarlatina are very commonly arousers of the scrofulous process, not only by the temporary impairment of health which follows them, but also through the catarrhs which are usually present in both diseases. Vaccination has often been accused of imparting scrofula; and, although this is untrue, since scrofula cannot be imparted in the sense of transference from one person to another, there can be no doubt that the predisposition may be roused into activity by the slight impairment of health associated with vaccination or by the slight injury inflicted at the point of introduction of the vaccinal virus. In some cases the disease has manifested itself for the first time during pregnancy or lactation, and there is no doubt that in cases where the disease has existed in childhood these conditions often cause it to reappear. In conclusion, it must be said that many cases apparently occur spontaneously--"the disease came on of itself"--or if there are any exciting causes they were so trivial as to have escaped notice altogether.

Finally, it must be remembered that the eczemas, catarrhs, ophthalmias, otitis, chilblains, erysipelas, and numerous other local disorders of nutrition which are often the causes of graver manifestations of the disease, are themselves very prone to run a peculiar course characterized by chronicity and intractableness; and many regard these disorders as themselves manifestations of scrofula. Indeed, Virchow, basing his argument upon the fact "that scrofulous enlargement of the lymphatic glands of the neck often follows upon certain diseases affecting the throat, such as mumps, diphtheria, and scarlet fever, maintains that scrofulous proliferation of these glands, like ordinary inflammatory hyperplasia of the same organs, is always secondary to some peculiar process going on at the mucous surface or other part which is in direct relation with them by means of the lymphatic vessels; that scrofulous disease of the glands of the neck is traceable to some inflammatory condition of the throat, fauces, or contiguous parts; of the bronchial and mediastinal glands, to pulmonary or bronchial inflammation; and of the mesenteric and retro-peritoneal glands, to similar conditions of the alimentary canal. But he considers that there may be some special element or quality in the primary inflammation, and a tendency in its products to undergo rapid decay similar to that which characterizes the morbid products of the diseased lymphatic glands, but that generally they are not recognizable, from the fact that in this case the cells are mostly developed at a free surface, and are speedily shed from it." He admits, however, "that there may be some special aptitude or weakness, congenital or acquired, in the lymphatic glands of certain persons, or of certain parts of them, which makes their inflammations, induced by indifferent causes, assume the scrofulous character."[6] From the last of these propositions no one will be likely to dissent, but that there is "some specific quality or {238} element in the primary inflammation," etc. few will be inclined to agree. There is nothing peculiar in the eczemas, ophthalmias, catarrhs, etc. spoken of, except that they occur in persons possessing the scrofulous diathesis; and it is this alone that gives them their special characters, if there are any. In other words, we cannot say that these disorders occur in certain children because they are scrofulous, but that they are specially intractable on account of the scrofulous diathesis upon which they are engrafted. Millions of children have catarrh and ophthalmia (indeed, few escape these disorders throughout the first ten or twelve years of life) who never show any other evidence of the scrofulous taint; and nearly all have measles and scarlatina, but it is only the scrofulous who usually suffer severely from the secondary effects of these diseases. But they do not have measles or scarlatina because they are scrofulous, and we can with no more justice say that they have catarrhs or other inflammations because they are so. We do not believe, therefore, that strumous children have cutaneous and catarrhal inflammations simply because they are strumous; and if we speak of scrofulous catarrh or ophthalmia or eczema, we use the terms in the same sense as when we would speak of a scrofulous measle, scarlatina, or whooping cough.

[Footnote 6: The Theory and Practice of Medicine, by Jno. Syer Bristowe, M.D., 1879, pp. 80, 81.]

Although we have few reliable statistics bearing upon the question, it can scarcely be doubted, judged by the results of casual observation, that scrofula is much less frequent in America than in Europe, and that in the latter there is less of it than formerly. The cheapness of land in America has prevented that excessive overcrowding that exists in the older and more densely populated countries, and the abundance and cheapness of animal food has prevented that excessive feeding on bread and potatoes which constitutes such an important factor in the production of the scrofulous diathesis in some other countries.

PATHOLOGY AND MORBID ANATOMY.--The most important and central anatomical and pathological facts both in the causation and progressive development of struma, according to the writer's views, are--

1st. That faulty anatomical--or rather histological--construction of the tissues of the scrofulous individual already alluded to as having been first brought to the notice of the profession by Formad of Philadelphia, which consist of an unusually large number of lymph-spaces (which are also unusually large), and consequently an excessive number of lymph-vessels and lymph-glands.

2d. Excessive production of rudimentary lymphoid cells, and probably also of lymphatic tissue.

3d. Diminished and insufficient number of the capillary blood-vessels; and, as a necessary consequence of these,

4th. Diminished nutritive activity of all those processes, both physiological and pathological, which depend upon a full supply of nutritive blood.

The most striking feature in all scrofulous inflammation is excessive cell-growth, but these cells show little tendency to differentiation and organization, probably for two reasons: 1st, because they are derived from the blood-vessels principally, and not from proliferation of the proper connective-tissue cells of the part; and 2d, because they are insufficiently supplied with nutrition from the scanty blood-vascular {239} network, and this supply is too rapidly absorbed into the lymph-spaces, and is carried off by the too numerous lymph-vessels. The cells, therefore, speedily perish, undergo partial or imperfect fatty degeneration, and finally caseation, unless the process is going on at a free surface, in which case, of course, they are shed and thus gotten rid of.

Virchow some time ago called attention to the predominant cellular character of the scrofulous exudation and the low vitality of the cells which compose it. Rindfleisch declares that the fresh scrofulous exudations contain relatively large cells with glistening protoplasm, and that the white blood-corpuscles have a tendency in scrofulous persons to grow larger on their way through the connective tissue. He adds that they swell up by the imbibition of albuminous substances, and by this very swelling die and slowly degenerate.

It seems to the writer, however, that it is probable that herein lies the reason why swelling and apparent hyperplasia of the lymphatic glands in the neighborhood of a local inflammation occurring in a scrofulous person always takes place. The swollen cells become arrested at the first gland they reach, and block the channels through the gland. Successive additions of cells continue to block these channels, and finally the passage of lymph through the gland becomes impossible, and then begins that secondary increase of the lymph-cells in the gland resulting from their inflammatory proliferation.

"In scrofulous inflammation," say Cornil and Ranvier,[7] "there is a remarkable tendency to permanent infiltration of the affected tissue. In simple inflammation (i.e. inflammation in non-scrofulous persons) the infiltration is a temporary condition which terminates in suppuration, in organization, or in resolution." Now, the several steps in this process of resolution are--contraction of the distended blood-vessels, thus cutting off the excessive supply of blood which has caused the exudation and cell-proliferation; fatty degeneration of the new cell-formation; liquefaction of this fat by union with the alkaline blood-plasma, converting it into a dialyzable (saponaceous) liquid which can now be readily absorbed by the veins. In scrofulous infiltration the cells are speedily attacked by fatty degeneration (which seems to be strictly a physiological process), but instead of becoming liquefied, it (the fat) remains, slowly dries and hardens, and finally becomes converted into the so-called cheesy mass or cheesy infiltration. It does not liquefy, because it does not receive a sufficiently abundant supply of the alkaline blood-plasma from the scanty blood-vessels, and that which is supplied too rapidly flows into the numerous large lymph-spaces and is carried off by the lymph-vessels. In the case of the infiltrated gland the supply of this plasma is cut off in both directions. The passage of lymph through the gland is blocked, when, of course, none can then reach it through the lymph-vessel leading to it, while the swelling of the gland itself from accumulated cells compresses the neighboring nutrient vessels and cuts off the supply from this direction also. Hence the speedy death, fatty degeneration, and caseation (not liquefaction) of the cells.

[Footnote 7: Ed. 1880, p. 114.]

"The newly-formed material not only interrupts the lymph-passages of the gland, but also compresses the blood-capillaries in such manner that the circulation completely stagnates. It is impossible by any {240} method of injection to penetrate into the most swollen parts of the gland. With the supply of blood the nutrition also self-evidently ceases; the gland falls into caseous degeneration. Where this enters in the gray mass first becomes opaque, then whitish-yellow, non-transparent, dry, friable. If the whole gland has passed into the caseous condition, it appears upon a section as a fresh potato, only not quite so moist, but just as homogenously yellowish-white."[8]

[Footnote 8: Rindfleisch, Textbook of Pathological Histology, 1870, p. 202.]

The subsequent fate of these glands seems to depend somewhat upon their situation. In the mesenteric and bronchial glands almost always the caseous mass is attacked by calcareous infiltration, and finally dries into a solid chalky concretion. The writer counted seven of these chalky masses around the primary bronchi of a boy about fifteen years old whose body was brought into a dissecting-room in Baltimore City. But the more common result of the caseous process in the glands of the neck is softening. "The caseous dépôt melts from within outward into a whitish-yellow, whey-like fluid, which holds a fatty granular detritus suspended in smaller or larger fragments. If all the caseous material has softened, the neighborhood of the gland is wont to inflame; this inflammation facilitates the way for the scrofulous pus outward. This is evacuated, and we have the scrofulous ulcer, with its overhanging, bluish, hyperæmic, flabby edges. At length this opening also closes, and a drawn-in, radiated cicatrix marks the place where the evacuation took place."[9]

[Footnote 9: Ibid., loc. cit.]

But it must be borne in mind that all so-called scrofulous hyperplasias of lymphatic glands do not run this destructive course. Undoubtedly, in a few cases there remains a sufficient nutritive supply to carry on the liquefactive process which normally follows fatty degeneration, and thus resolution of the affected gland takes place. We are, however, of the opinion that Virchow was mistaken when he asserted that complete resolution of the cheesy material could take place; and from what we know of the dangerous and usually fatal consequences of the absorption of this cheesy detritus, Rindfleisch is certainly in error in describing this as the most desirable possibility of decomposition.

We have heretofore purposely avoided any mention of tubercle or tuberculosis as a part of the scrofulous process. In the views of many physicians the relations between the two processes are so close that to them tuberculosis and scrofulosis mean one and the same thing. While medical opinion as to the true meaning of the word tubercle was so discordant and unsettled, while so many products of diverse pathological processes were included in that term, and while many, following the view of Burdon-Sanderson of England, believed that tubercle always takes its origin in small, even microscopic, collections of lymphatic tissue, such a belief in the identity of the two processes was not only possible, but reasonable. But since, by very many good authorities, the term tubercle is now limited to the miliary or submiliary tubercle, since numerous inoculation experiments have shown that tuberculosis can be induced in non-scrofulous animals, and Koch of Germany has proved that there exists in decaying tubercle a peculiar and distinctive bacillus which even when cultivated out of the body of a tuberculous person will excite tuberculosis also if inoculated upon a non-scrofulous animal,--a belief in the identity of the two diseases seems to be no longer tenable. Certainly, it {241} would seem that to Sanderson's view that tubercle always takes its origin in lymphatic tissue it is only necessary to reply that the subjects of miliary tuberculosis do not more frequently than those suffering from other non-scrofulous diseases present those larger glandular hyperplasiæ which are so distinctly characteristic of scrofula, and to which many persons limit the term scrofulosis. It may be said, perhaps, that the converse of this is not true, and that scrofulous persons are more frequently attacked by miliary tuberculosis than an equal number of non-scrofulous persons. But the extreme susceptibility or liability of the scrofulous to be attacked by numerous and even diverse morbid processes, and the profound cachexias and dyscrasias which the scrofulous processes engender, amply account for the apparent susceptibility of the scrofulous to be attacked by miliary tuberculosis. The strumous are more susceptible to the exciting causes of tuberculosis undoubtedly, but perhaps the same may be said in regard to measles, scarlatina, and the various other exanthemata. It is undoubtedly true also that among the lower animals (and probably also in the higher ones) the introduction into the circulation of the semi-purulent fluid resulting from the breaking down of a cheesy scrofulous gland will produce that peculiar (perhaps specific) dyscrasia which results in miliary tuberculosis. But as it has also been abundantly proved that a similar fluid derived from a cheesy pneumonia, or from the inflammatory products of any other disease which have undergone the cheesy degeneration, will also excite tuberculosis, the fact does not seem to tell in favor of the identity of, or even of any close relationship between, the two processes. Still, as the scrofulous more frequently than other people are the subjects of this cheesy process, it is not surprising that they should more frequently be poisoned by the entrance into their blood of the cheesy detritus.

We do not deem it necessary to adduce all the evidence or to state authorities upon this subject, but we think we are justified in stating the following doctrine in regard to the relation of scrofula to tuberculosis as best supported by facts and by the consensus of medical opinion:

Scrofula is a purely diathetic disease inherent in the individual.

Tuberculosis is a cachectic (possibly a purely dyscratic) one, not inherent in the individual, but always caused by some morbid influence from without. Tuberculosis may therefore occur in the non-scrofulous as well as in the scrofulous.

But the scrofulous are more likely than others to have tuberculosis--1st, because of their greater susceptibility to all morbid influences; 2d, because the scrofulous processes are apt to produce some cachectic condition which is always a condition precedent to tuberculosis; and, lastly, because the products of decay resulting from scrofulous processes may enter the circulation and directly produce the tuberculous dyscrasia. These remarks of course apply only to primary tuberculosis.

But while we thus deny anything else than a purely incidental relation of scrofula to tuberculosis, we believe that there exists the very strongest possible relationship of scrofula to pulmonary consumption. We think we are justified in stating that fully 95 per cent. of all cases of pulmonary consumption are of inflammatory origin, and of that variety miscalled catarrhal pneumonia. Broncho-pneumonia or catarrho-pneumonia more exactly describes the process. It begins as a catarrh, {242} sometimes in the nasal passages or post-nasal fossæ, sometimes in the pharynx, but most frequently in the trachea and large bronchial tubes, and sometimes rapidly, but oftener more slowly, travels downward and invades the lining membrane of the air-sacs, which soon become packed with cells derived partly from emigration of leucocytes, partly from proliferation of the epithelium lining the sacs. These cells soon undergo the cheesy degeneration, and, finally breaking up, as in the case of the scrofulous gland, cause the formation of vomicæ attended with the familiar signs of pulmonary consumption. Every step in this process is attended with that abundant cell-production, and the process itself is marked by that inveteracy and intractableness, which always characterize scrofulous inflammations, or rather inflammations in the scrofulous. Occurring as they most frequently do in young adults, these cases are often mistaken for pulmonary tuberculosis; and as post-mortem examination generally reveals a more or less abundant secondary tubercular eruption caused by absorption of infective material from the centres of cheesy degeneration and softening, the diagnosis is claimed to be confirmed. But they are for the most part, nevertheless, cases of genuine scrofulous inflammation of the bronchial membrane and lining membrane of the alveoli, and should be called scrofulous pneumonia. It is true that Mr. Phillips, Mr. Kiener, Villemin, Grancher, Mr. Treves, and others have collected numerous statistics which would show that comparatively few of those who had died of pulmonary phthisis bore any evidence of previous scrofulous disorder. But as the principal evidence relied upon to prove this fact was an absence of scars resulting from suppurating glands, their statistics are inconclusive. Besides, it is a well-known fact that there is a decided antagonism between scrofulous diseases of all kinds, and a patient who has one severe or well-marked manifestation of scrofula is not likely to develop another strumous disease at the same time. The records of the Margate Infirmary for Scrofula show this fact very strongly, and numerous writers--among whom may be mentioned Holmes, Birch-Hirschfeld, Walsh, Mr. Treves, and others--strongly express the same opinion. Indeed, some of them go so far as to maintain that one form of the scrofulous manifestation confers protection against others. The question may perhaps be more clearly stated by saying that the scrofulous, like the non-scrofulous, have their special predispositions and indispositions to certain morbid affections, and while one scrofulous child may be specially predisposed to affections of the bones, joints, skin, or other tissues, it may have no predisposition whatever to affections of the lungs or lymphatic glands, etc. This difference in vulnerability or invulnerability of certain tissues or organs in individuals, whether scrofulous or not, is so distinctly recognized as a controlling factor in determining the special form of disease resulting from a given irritant that its discussion is entirely unnecessary. It is argued against the identity of scrofula and pulmonary consumption that the commoner manifestations of the former occur in childhood for the most part, while consumption is a disease of adult life. But this is readily accounted for by the different morbid tendencies and exposures in the two periods of life. "Scrofula tends to appear in early life on account of the unusual activity of the lymphatic system at that period, and phthisis somewhat later--at a time, indeed, when the lungs are in more active use, when {243} sedentary and perhaps unhealthy pursuits are assumed in exchange for the liberty of childhood, when the modifying influences of puberty are active, and the structural responsibilities of adult life press heavily on an organization never other perhaps than frail.... I would, on the contrary, assert that scrofula and phthisis are as much manifestations of the same morbid change as acute bubo, acute orchitis, and acute pneumonia are outcomes of one single process--acute inflammation."[10]

[Footnote 10: Scrofula and its Gland Diseases, by Frederick Treves, F.R.C.S., Eng.; New York, 1882, p. 62.]

It is entirely unnecessary--and indeed it would be too tedious--to describe the anatomical appearances of the almost innumerable lesions met with in the scrofulous. Holding as we do that scrofula is not a disease per se, but merely a condition resulting from malnutrition and consequent faulty construction of the tissues during the early years of childhood, no peculiar or distinct anatomical lesion can be ascribed to it; and yet every lesion of nutrition as well as of function may have certain specific characteristics impressed upon it by the scrofulous diathesis. These may be briefly summed up as great slowness in evolution, intractableness, incurability, and chronicity of all pathological processes, and in all inflammatory processes abundant cell-production and tendency to caseation.

SYMPTOMS, COURSE, DURATION, AND TERMINATIONS.--A great deal of fine writing has been expended in describing the physiognomy of scrofula, and for ages writers exercised their descriptive powers upon the type of face and form supposed to be indicative of the disease. It is almost needless to say that much of this has been evolved from the imaginations of the writers, while many of these descriptions are not pictures of those liable to suffer from scrofulous processes, but of those who are already the subjects of these, and are simply types, not of the scrofulous diathesis, but only of the scrofulous cachexia. Many of these pictures, too, were drawn not from the scrofulous, but the tuberculous patient, because they were considered identical. Scrofula is not confined to the dark or the fair, the dull or vivacious, nor even to the weak and puny or the strong and robust; but all these may have this faulty and often fatal construction. Nor do we believe that scrofulous children are either more brilliant or more stupid than other children. At most we can only say that the scrofulous habit is marked by a deficiency of blood and a bad nutritive state of the more important and more highly organized tissues. In some an abundance of fat is found, giving to the individual a certain amount of plumpness, which might be thought to be inconsistent with a state of bad health; in others there is an imperfect development not only of the subcutaneous fat, but of the skin and muscles also, so that they appear tender and delicate. In the first of these conditions there is supposed to be an indolent state of the processes of constructive and destructive assimilation; in the second, an unnatural activity of these processes. These differences have led to a classification of scrofula into the phlegmatic or torpid and the sanguine or erethistic forms, which Canstatt has thus described: "An unusually large head, coarse features, a thick chin, a swollen abdomen, enlarged cervical glands, and flabby, spongy flesh." The erethistic form is said to possess "a skin of remarkable whiteness, with a tendency to redden easily, and through which the {244} rose-pink or bluish subcutaneous veins are visible, a deep redness of the cheeks and lips, blueness of the thin and transparent sclerotica, which imparts a swimming and languishing look to the eyes. The muscles of such persons are thin and soft, and their weight is light in proportion to their stature, indicating a slightness of their bones. The teeth are handsome and of a bluish lustre, though long and narrow; the hair is soft."[11] Although this description may be characterized as diagrammatic, since it describes rather the extremes and not the mean of the general appearance of the scrofulous, and numerous cases will be met with that cannot be assigned to either of the above categories, yet as quite a large number of cases will be seen that obviously belong to one or the other of these types, and as, moreover, we shall see that by this classification we shall obtain valuable data for therapeutic indications, it may be well to preserve this division of the scrofulous into the lymphatic and sanguine types.

[Footnote 11: Niemeyer's Text-book of Practical Med., vol. ii. p. 741.]

The leading points in the physiognomies of each of these types were admirably shown in the composite photographs exhibited by Dr. Mohamed at the last International Congress in England. By some special process a composite photograph of many faces was, as it were, condensed into a single picture, in which all that is common remains, all that is individual disappears. And although Mohamed's pictures were all of phthisical patients, it must be admitted that the two types of coarse struma and sanguine struma were strikingly illustrated, and were very suggestive of Canstatt's descriptions as given above. But it must be borne in mind that a large number of the strumous belong strictly to neither of these types, but rather to a medium between the two. "Such a type would include what is known as pretty struma. The general features of the individuals so termed belong to the so-called phlegmatic type, but the coarseness of the features is toned down; the lips would be called full, not tumid; and a coarse flabbiness would subside into a pretty, plump condition of the body. The limbs, if not actually graceful, are at least prettily rounded. The skin may not be thin and fine, but it is soft, white, and clear. The general expression is not absolutely apathetic, but would be termed gentle and eminently feminine. Excellent representations of this type of pretty struma were also shown in the photographic series above mentioned."[12]

[Footnote 12: Treves, Scrofula and its Gland Diseases, p. 84.]

This matter of physiognomy of the scrofulous has this much at least of practical importance--viz. that to the sanguine or erethistic type belong those cases that show distinct heredity, while the phlegmatic or torpid is usually the type assumed in the acquired forms. While there are doubtless numerous exceptions, it will generally be found that scrofula in the rich assumes the first, and in the poor the second, of these forms. It has been asserted that the erethistic form is more apt to develop tuberculosis or phthisis; and to a certain extent this is doubtless true, but the torpid are by no means exempt from this grave accident. The first are undeniably more liable to the more severe and fatal forms of the disease, which run a more rapid course and are less amenable to treatment, while in the second phthisis is more apt to be chronic and incomplete recoveries are by no means rare. The first form is said to {245} be more frequent in women, while the second is more frequent in males; and this accords with my own observation and experience.

There are certain features more or less peculiar to scrofula, besides those appertaining to the general physiognomy already discussed, which it may be well to call attention to, since these may aid us in detecting the scrofulous diathesis even before the grosser manifestations have declared themselves.

Allusion has already been made to the defective blood-vascular capillary network in the scrofulous as a necessary consequence of the excessive predominance of lymph-spaces and lymphatic vessels. Indeed, there can scarcely be a doubt that the slowness of evolution of various pathological processes, their chronicity, and the absence of tendency to resolution and cure of inflammatory lesions, so prominent a feature in all scrofulous manifestations, is due to this very condition. It is especially in the coarser type of struma that these defects in the circulation are most conspicuous. In these the pulse is often below the average, soft, and wanting in vigor. The cheeks and limbs often assume a bluish and mottled aspect, due perhaps to a tendency to stagnation of the blood in exposed parts. The extremities appear swollen as if from cold, and in the winter generally appear chapped. They are particularly liable to chilblains, which persist far into the summer and often take on a very unhealthy action. This last feature is so common as to constitute an important symptom in scrofula. These defects in the circulation also probably explain the frequent catarrhs and eczemas with which such persons are affected, and account also for their intractableness as well as the unwholesome character of their wounds.

For the same reason (deficient circulation) the temperature is generally found to be a little lower in the coarsely strumous than in healthy children, and even in their fevers a very high temperature is rarely met with. Acute sthenic inflammations are rarely seen, and hence these persons seldom have acute croupous pneumonias; it is rather the catarrhal variety, and of this the subacute and chronic forms, which they suffer from.

Opinions are completely at variance as to the influence of the scrofulous habit in delaying or hastening menstruation. Lugol referred to the frequency of dysmenorrhoea among the strumous, and there is no doubt that the scrofulous as a rule often suffer from suppressed or scanty menstruation. But it is improbable that the diathesis exerts any influence whatever in determining the period of puberty in either sex.

We have already stated our belief that the strumous are neither more intelligent nor stupid mentally than other people. An exception ought perhaps to be made to this in the case of the exaggerated type of the coarsely strumous. In these extreme cases we must confess that we have generally found associated great slowness and dulness of the mental faculties. If great intelligence and precocity are sometimes met with, it is only in the erethistic or pretty struma, who, because it is the delicate one of the family, is petted, has more notice taken of it, and afforded every facility for the development of the points that make up the precocious infant. The prettiness of these children, moreover, attracts more attention to them than to other children or than the bulk of the sickly would receive.

In young scrofulous children we often observe a considerable amount {246} of close-lying downy hair upon the forehead, more abundant upon the sides of the forehead. Upon the arms and back from the occiput to below the shoulders also a like condition is often seen. Later the eyelashes appear thicker and longer, and the eyebrows more abundant, coarser, and longer, than in the non-scrofulous. The color of these is also apt to be darker than the rest of the hair.

Constantine Paul, as quoted by Treves, has drawn attention to certain changes in the ears, after they have been pierced for earrings, that he considers to be diagnostic of scrofula. The mere weight of the earring seems to cause the puncture to slowly ulcerate, and the ring thus cuts its way out, either leaving behind it a linear scar or a slit in the lobule. If the lobule be repaired the ring may cut its way out again, and this may occur three or four times. These changes seem not so frequently to be observed in England and America, and may be due in part to the fact that earrings of greater weight, and more frequently of base metal, are worn in France than in the countries named. But still, from what has been said concerning the histology and minute anatomy of the scrofulous, and the consequent less resistance of the tissues, this cutting-out process by earrings is just what we would be led to expect in strumous persons.

The thick upper lip is never absent from the older descriptions of the physiognomy of the strumous. This is almost invariably present in the coarse type of struma, and seldom absent even in the erethistic. It is not always due to irritation from acrid discharges from the nose, as is maintained by Treves, though doubtless the eczematous and herpetic eruptions are often caused and maintained by these discharges, and these may in time cause and increase this thickening.

The teeth in scrofula show nothing that is distinctive, though there is undoubtedly a tendency to early decay. As this tendency to decay is, however, so common in many persons who have at least shown no other evidences of the scrofulous diathesis, no positive conclusions can be drawn from this fact.

Clubbed fingers, too, so common in persons who have become cachectic from the long persistence of scrofulous disorders, are not characteristic. Clubbed fingers and incurvated nails will generally be found in persons suffering from any disease characterized by slow wasting. They are seen in phthisis of all varieties, as well as in cancer, heart disease, aneurism, Bright's disease, empyema. They therefore have no significance as far as struma is concerned.

GENERAL MANIFESTATIONS OF SCROFULA.--As, according to our view, there is no such disease per se as scrofula, but simply a diathesis which impresses its own malign influence upon every other disease with which the strumous individual may happen to be afflicted, increasing perhaps the general predisposition to be injuriously affected by all morbific influences, or impairing the powers of resistance to these, and especially intensifying any special predisposition which age, sex, personal peculiarities, occupation, habits, mode of life, or heredity may have created, we cannot describe any morbid processes as specifically scrofulous. At most, we can only say that struma is more apt to impress its malign influence upon certain diseases or upon inflammations and injuries of certain tissues, that some diseases in the scrofulous are more apt to be {247} attended by certain complications and followed by certain sequelæ, and that all of these are characterized by chronicity and incurability, by slowness of evolution of pathological processes, and, in the case of inflammations, by a tendency to profuse cell-production and to rapid caseation. Thus, measles is apt to be complicated with or followed by otorrhoea, chronic bronchitis, caseation of bronchial glands, phthisis, and even tuberculosis; scarlatina by otitis, hyperplasia of the tonsils, caseation or suppuration of the submaxillary and other lymphatic glands about the neck, and by chronic catarrh of the renal mucous membranes, causing dropsy and finally death; eczemas about the face or catarrhs of the mouth and throat by hyperplasiæ and caseation of lymphatic glands in the neighborhood. Boils and other subcutaneous inflammations of the areolar tissue, so common in childhood and adolescence, do not run their usual rapid course, ending in suppurations and cicatrization, but become in the one case the scrofulous gumma, degenerating into the scrofulous ulcer, or if more deeply seated become a cold abscess. A single injury of a joint, whether mechanical or rheumatic, will "sometimes take the form of a simple hydrarthrosis, sometimes that of a so-called tumor albus, while at others it assumes the nature of a malignant arthrocace, accompanied by suppuration, caries of the articular surfaces, burrowing of pus, and the establishment of fistulæ."[13] A slight injury inflicted in the sports of childhood and soon forgotten--the prick of a pin perhaps--is followed by a disease sometimes beginning in the periosteum, sometimes in the bone itself, and presenting at one time the character of periostitis and ostitis, and at another that of caries or necrosis, or of the two combined.

[Footnote 13: Niemeyer, loc. cit.]

"As long as the existence of cheesy masses," says Niemeyer, "was regarded as characteristic of the tuberculous nature of a disease, it was of course necessary to ascribe many of the inflammations of the joints and bones of scrofulous persons to a complication of scrofulosis with tuberculosis."[14]

[Footnote 14: It is a well-established fact, however, that true miliary tubercles are often found in the neighborhood of bone and joint affections in the scrofulous, as well as in lupus, in cold abscess, and in softening caseous glands, which last are considered by many as specifically scrofulous diseases. It is suggested that an explanation of this may be found in the probable fact that caseous pus may be capable not only of producing a general tuberculosis when carried by veins or lymphatics into the blood, but that it may also set up a local tuberculosis by a morbid influence exerted upon the neighboring lymphatics and blood-vessels with which it may come in contact. We are aware that Wilson Fox (according to the Medical Times and Gazette), captivated by the theory of Koch, has recently recanted his belief in the inoculability of tuberculosis with anything except tubercle. But we are afraid that Dr. Fox (who we believe was one among the first to confirm Ferdinand Cohn's experiments in producing tuberculosis in rabbits and guinea-pigs by inoculating them with caseous pus) is suffering from that most active and virulent of all contagions, the contagion of popular belief. Just now a belief in specific bacilli and micrococci may be said to be riding upon the crest of a very high wave of popularity, and we are afraid that many of those who are rushing forward to mount this wave also will ultimately find themselves stranded upon that shore which has been strewn with so many wrecks in the past.]

A simple bronchitis, possessing nothing specific in its origin at least, will persist and extend to the lobuli of the lung and excite a catarrho-pneumonia which ends in consumption and death; a simple intestinal catarrh will result in inflammation and caseation of the mesenteric glands--a tabes mesenterica; or a simple dysentery, persisting in spite of the most {248} approved treatment, causes proliferation and caseation of the endothelial cells of the follicles, terminating in that obstinate and intractable follicular ulceration which wastes the strength and wears out the life of the little patient.

A simple conjunctivitis of the globe often will be followed by ulceration of the cornea, giving rise to intense photophobia, and leave behind it opacities of that organ which remain a perpetual evidence of the scrofulous diathesis, if they do not shut out for ever the light from the eye. Or if it is the palpebral conjunctiva that is affected, the meibomian glands and follicles of the ciliæ become involved, destroying the lashes and leaving the lids raw and everted or inverted--a perpetual deformity. In short, there is no conceivable disease or injury occurring in what we may call the intensely scrofulous that does not have impressed upon it some one or more of the malign characteristics which we have spoken of as indicative of the scrofulous diathesis. But it is not probable that there is ever any special disorder or lesion which can be said to be caused exclusively by scrofula; or, in other words, there is no such disease as a specifically scrofulous one. Lupus, cold abscess, and particularly caseous glands, are especially attributed to struma, because they are often thought to make their appearance independent of any assignable cause; but as boils, eczema, impetigo, and numerous other affections of the skin and areolar tissue affect children who are not scrofulous, and equally independent of any known causes, the argument is not conclusive. Besides, all these affections occur sometimes in the non-scrofulous; and even caseation of a single inflamed gland quite often occurs in children who are weak or in ill-health, but who show no other evidences at that or at any other time of the scrofulous habit.

DIAGNOSIS.--The only affection likely to be mistaken for scrofula is congenital or acquired syphilis in its later manifestations. In this disease we see the same tendency to increased cell-production, the same tedious, slow, and intractable inflammations and ulcerations, which are characteristic of scrofula. And this apparent similarity has induced many persons to believe that scrofula is nothing else than syphilis in the second or third generations. But in congenital syphilis the lesions usually make their appearance soon after birth or are present at birth, and long before even hereditary scrofula begins to show its malign influence. In most cases, too, a history of syphilis can be obtained, and even when this is not obtainable a few inunctions or fumigations with mercury, in connection with a few large doses of iodide of potassium, will very quickly decide the question of diagnosis for us.

In the case of lupus, in which Erichsen admits there is no means of positively distinguishing the syphilitic from the so-called scrofulous varieties, the diagnosis is more difficult. But as this disease appears later in life than the more ordinary scrofulous manifestations--when, therefore, a history of syphilis can generally be obtained if there is one, and when there would almost certainly be also a history of scrofula if it existed--it would seem that the diagnosis even in this case cannot be so difficult. Diagnosis here, however, is of little consequence, since the treatment recommended for both forms is the same.

PROGNOSIS.--This of course depends upon the nature of the special lesion. The simpler lesions incident to childhood, such as glandular {249} hyperplasiæ, catarrhs, eczemas, impetigoes, etc., usually do well under appropriate treatment and proper hygienic conditions. Diseases of joints, bones, mesenteric glands, etc. often terminate fatally or result in serious deformities and permanent impairment of function. Not infrequently diseases of the bones and articulations, attended with profuse and protracted suppuration, cause amyloid degeneration of the liver, kidneys, spleen, or other glandular organs, and, as a consequence, death. Catarrho-pneumonia in a scrofulous subject almost invariably causes phthisis sooner or later. Occasionally the caseated cellular exudation in the air-sacs remains quiescent for months, and even years, the patient remaining quite well except for a harassing cough during the winter months; but sooner or later the caseous mass will soften, the symptoms of active consumption ensue, with fever and wasting, and death closes the scene. Far more frequently, however, softening and suppuration follow swiftly upon the caseous degeneration, and the whole process occupies a period of only a few months. Tuberculosis especially runs a rapid course in these subjects, and while a few perhaps only develop tuberculosis of the lungs--in which case the duration of the disease may be a little longer--in by far the larger number there is a generalization of the tubercular process which puts a speedy end to their existence.

TREATMENT.--This may be most profitably discussed under two heads--prophylactic and therapeutic.

Prophylactic.--Scrofulous persons who are closely related by blood should be earnestly advised not to intermarry. We have so often seen the deplorable results upon offspring of such marriages that we cannot too strongly urge this upon the profession. Such persons should be frankly and clearly told what are most likely to be the consequences of such marriage, and all possible moral influences should be exerted to prevent them. The canons of the Church wisely interdict such marriages, but, unfortunately, its ministers seldom attempt to enforce them, or if they do their efforts are made ineffectual by the facility with which the marriage-rite can be obtained from civil officers in most of the States of the American Union. The medical profession can do more than any other class to diffuse knowledge and create a correct public opinion upon this subject, but, unfortunately, it too often neglects this important mission.

The children of scrofulous parents should be nursed (at the breast) longer than other children, so as to ensure an abundance of animal food during the first two years of life. Some advise scrofulous mothers not to nurse their children, lest they should imbibe the scrofulous taint through the milk. This fear is entirely groundless. We know of no reason why such a mother should not nurse her offspring, unless it be that it injures her. The child receives its scrofulous inheritance not through the mother's milk, but from the ovarian or spermatic cell. Milk can convey no disease or diathesis except on account of its deficiency in nutritive properties. If, therefore, there is any special reason why the mother should not nurse her infant on her own account, it may be well to turn it over to a healthy wet-nurse; but the temptation to give an infant raised on the bottle starchy foods prematurely is too strong generally to be resisted. The numerous infant foods advertised consist principally of starch, and young infants would infallibly starve on any or all of them {250} if their venders did not always direct that they should be taken with a large quantity of cow's milk. If the circumstances of the parents do not enable them to obtain a wet-nurse, then good cow's milk constitutes the best food for infants until they have cut their canine and anterior molar teeth. The custom of weaning infants at a certain age in every case is a pernicious one. Some infants are as well developed as to their digestive organs at fifteen months as others are at thirty, and the eruption of the teeth may generally be taken as a safe guide as to that question. A moderate amount of food containing starch after the period indicated may be allowed, but always with a preponderance of animal food. It is not so much the starch that acts injuriously upon the nutrition of children as the excess of that substance; and if the food contains but little nutrition in proportion to its bulk, it is so much the worse. Even milk containing too little casein and fat in proportion to the watery elements may be perhaps quite as injurious as potatoes. And hence if the mother's milk should be poor in these elements, it ought to be supplemented with cod-liver oil or other animal fat in small doses.

A practice existed among the Southern slaves (and to some extent also among the whites) before emancipation which at first I was inclined to condemn until I saw the excellent effects resulting from it. Within an hour or so after birth a piece of fat salt pork or bacon was placed between the child's lips, and it was permitted to suck this at all times when not nursing. Tied to its wrist by a short string, so as to prevent swallowing it, this piece of pork furnished both nutrition and amusement to the infant for many hours while the mother was at work in field or garden. The children throve well on it, and thus treated we found them to be as well developed at twelve months as most other children were at twenty. It was doubtless due in part to this practice that there was so little scrofula among them.

An abundance of pure air is also a valuable factor in preventing the establishment of the strumous diathesis. Strict regard, therefore, should be had to ventilation, and overcrowding should if possible be avoided. Children over twelve months of age should not even be permitted to sleep with their parents, but should have in cold weather a crib, cradle, or other bed to themselves; and in warm weather they should be put to sleep in a net hammock, which is now so cheap as to be within the means of almost everybody. This will not only secure to them a better supply of air, but it will also prevent them from suffering so much from the heat, which is so potent a factor in the production of cholera infantum.

Bathing in proper season is also useful as a prophylactic. Sea-bathing especially has long enjoyed great credit as a remedy for scrofula, but we think this is often resorted to too soon and practised at improper times. In warm countries a bath of cold water may be taken every day in the year, but it should be given at the warmest hour of the day, not early in the morning. In all climates due regard should be had to the powers of resistance to cold and the promptness of reaction after the bath. If children remain cold and pale for a long time after the cold bath, the practice should be discontinued and tepid water substituted. In colder climates tepid bathing should be practised once or twice a day during the winter, and in summer a little lower temperature may be used. Bathing children under three or four years in the sea at any time is pernicious, {251} both because the temperature is too low and on account of the fright which it always causes in these young children. After four years a child will take to the water almost as instinctively as a young duck.

Therapeutic.--Almost all of the so-called scrofulous manifestations belong to the surgeon, dermatologist, or oculist and aurist, and we shall therefore say nothing about the special and local treatment of these manifestations, but refer the reader to works upon these several departments of medicine. But as little success will be had in the treatment of these special disorders unless due regard is had to the general condition, and unless the local treatment is supplemented by constitutional measures, we shall briefly give some directions for this constitutional treatment of the scrofulous individual.

It is important in determining upon the proper treatment in any given case to bear in mind the division of the scrofulous into the two types of torpid or lymphatic and sanguine or erethistic already described. It is true that in many cases it is not easy to determine to which class a patient belongs, and many possessing some of the characteristics of both certainly cannot be referred to either. Still, in many cases the discrimination is easy, and then furnishes very clear and valuable indications as to treatment. Iodine (and its preparations) has since the time of Lugol, who first brought it into prominent notice, been regarded as a useful remedy in scrofula. But burnt sponge (spongia usta), which contained the iodides of sodium and potassium, had been used to dissipate goitrous and scrofulous swellings many hundreds of years before the time of Lugol. It is a valuable remedy in certain cases, and if it is falling into disuse it is probably for the want of proper discrimination in the selection of cases. In all cases in which there seems to be an abundant production of fat, and therefore in nearly all the cases of coarse struma where there is an indolent process of assimilation and disassimilation, iodine and its preparations will be found useful. Indeed, in the form of syrup of iodide of iron we have rarely failed with it to cause strumous enlargements of glands to disappear when the remedy was used soon after their first appearance. Of course, neither iodine nor any other medicine can have any effect in removing these enlargements after the glands have become caseous. While good results may be obtained with the syrup in all forms of scrofula, it is unquestionably in the sanguine and neutral types that it is most useful. It should be given in doses of 10 to 30 drops to children under five years of age, and to older ones ½ to 1 fluidrachm three or four times a day may be administered. We have given the latter dose to children four or five years of age for a long time, with the best effect upon their scrofulous manifestations, and without any injury whatever to their digestive organs.

In the torpid types preparations stronger in iodine should be used. Here Lugol's solution or iodide of potassium or sodium will be found very useful, either alone or in connection with the iron preparation above mentioned. Indeed, as in these cases it seems to be disassimilation that appears to be specially faulty, even very small doses of mercury in the form of bichloride or biniodide will be found useful. Donovan's solution may be prescribed in these cases along with the active preparations of iodine with good effect, or if the arsenic in that preparation is objectionable, one-fiftieth of a grain of bichloride or biniodide of {252} mercury may be substituted. The mercurial should not, however, be continued longer than one or at most two weeks at a time, after which it should be suspended and the iodine continued.

Cod-liver oil, which is too indiscriminately prescribed in all cases, will be found to be of little use in the lymphatic types, if indeed it is not actually injurious; but in those cases with pale, thin skin, with deficient development of fat, and with small muscles--in short, those in which emaciation or delicacy is prominent--it is a most valuable remedy. It is almost surprising to see how rapidly ulcerations, caries, eczemas, catarrhs, etc. occurring in this class of subjects will disappear under the use of this medicine alone.

The hypophosphites and lactophosphates are also useful in this class of cases, especially where there is disease of bone or joints, in connection with the cod-liver oil. We have long been in the habit of using the following formula, which we have found very useful:

Rx. Pulv. Acaciæ, drachm ij; Ol. Amygdal. amar., gtt. vj; Syr. Calcii hypophosphit., vel Syr. Calcii lactophos., fluidounce iv; Ol. Morrhuæ, fluidounce iv; Ft. mist.

S. Teaspoonful to tablespoonful three times a day according to age. Syrup of iodide of iron may be added if desirable, though we prefer to give this by itself.

Gentle exercise, passive or active, pure air, well-ventilated sleeping apartments, a generous diet--in which wholesome animal food should predominate--and bathing are of course as necessary and as useful in the treatment as in the prevention of the scrofulous diathesis.

Alkalies should be given in all cases in which we are trying to dissipate enlarged lymphatic glands, for the reason that caseation of these glands occurs because of insufficient alkalinity of the blood to effect reduction of fat, and because also the strumous almost always suffer from excessive acidity of the gastric and other secretions. When the iodides of potash or soda or the hypophosphites of lime and soda are given, the additional administration of alkalies may not be necessary; but if not, bicarbonate of sodium or potassium (which have long enjoyed a good reputation in the treatment of struma) should be added to the other remedies.

Since the appearance of Niemeyer's Handbook of Clinical Medicine the proper treatment of scrofulous glands that have undergone the caseous degeneration has been a moot question. Some recommend the ablation of these glands by the knife, some advise spooning out the caseous matter through a small opening, while others prefer to await the natural process of softening and the discharge of the caseous matter by suppuration. There can be no question that the removal of these glands by the knife, when this can be done without serious risk, will leave behind a less unsightly scar, and will be attended with less fever and consequent deterioration of the general health, than usually attends suppuration. Spooning out the caseous matter will perhaps leave no extensive cicatrix, but we can never be sure that by this operation we have removed all the caseous matter, and it must certainly be more painful than the knife. Mothers will generally object to either of these {253} operations, and as the risks of infection by absorption of the caseous pus during the suppurating process do not seem to be very great, it is perhaps best to leave these glands to nature, unless the vitality of the patient is so low as to give reasonable ground for fear that the child may succumb to the effects of the natural process. If any surgical interference is deemed necessary, we are decidedly in favor of removing the caseous gland entire by the knife.

{254}

HEREDITARY SYPHILIS.

BY J. WILLIAM WHITE, M.D.

About a half century ago Mr. Abraham Colles, who had just resigned the professorship of the Theory and Practice of Surgery in the Royal College of Surgeons in Ireland, the duties of which, in the opinion of the college, he had discharged for thirty-two years in an "exemplary and efficient manner," wrote the following introductory paragraph to his remarkable chapter on "Syphilis in Infants:" "Perhaps there is not in the entire range of surgical diseases any one the contemplation of which is more calculated to arrest our attention or to excite our interest than syphilis infantum."

Although it was not then, and is not at the present day, strictly relegated to the domain of surgery, hereditary syphilis, like its parent disease, was generally treated of by the practitioner of that branch of medicine. And yet in the great majority of instances the management of such cases, especially as regards their family relations, the relations of husband and wife, the management of the latter during pregnancy, the delivery and subsequent care of the child, the necessary attention to the safety of other members of the family--in fact, all of the most weighty responsibility--falls upon the ordinary medical attendant. It is therefore in every way proper that the condition should receive some notice in a system of general medicine.

A proper presentation of the subject of hereditary syphilis involves a consideration of the vexed question as to the mode by which the disease is conveyed from parent to offspring. That it may be so transmitted has been generally believed since the doctrine was first announced by Torella at the end of the fifteenth century; and the facts in its support are so numerous and convincing that, in spite of a few distinguished opponents--among whom John Hunter was the most conspicuous[1]--it has been unhesitatingly accepted by the profession down to the present day. As regards the manner of transmission, however, controversy has been and still is rife. Opposing theories have been constructed and ardently supported, differing radically as to essential points, often resting upon exceptional or anomalous, and still oftener upon imperfectly observed, cases.[2]

[Footnote 1: Works of John Hunter, vol. ii. p. 383.]

[Footnote 2: Parrot, in a clinical lecture on syphilitic abortion (Le Progrès Médicale, Nov. 3, 1877, p. 798), says: "The infection of children was known, but its true origin was not suspected. The belief of Gaspard Torella (1498) and Matthioli (1536) that it came from the nurses through the milk was generally accepted."

{255} According to Diday, Paracelsus (1529) was the first to plainly state the heredity of syphilis: "Fit morbus hereditarius et transit a patre ad filium."

Others attribute the original announcement to Augier Ferrier (1553), and it seems certain that he was first to specify the three modes of infection of the product of conception: "La semence du père, celle de la mère, et la contamination de la mère durant la grossesse."

Fallopius in a posthumous treatise on the Mal Français (1566) adds the authority of his name to this view: "Præterea videbitis puerulos nascentes ex foemina infecta, ut ferant peccata parentum, qui vedentur semi cocti."

Ambroise Paré also acquiesced in the theory, saying, "Souvent on voir sortir les petits enfants hors le ventré de leur mère, ayant ceste maladie, et tost après avoir plusieurs pustules sur leur corps; lesquels étant ainsi infectés, baillent la vérolle à autant de nourrices qui les allaictent."

Subsequently, Mauriceau, Boerhaave, and Astruc sustained the same view, which, with the single exception of Hunter, had no prominent antagonist.

It was not, however, until the eighteenth century that it was described with any attempt at detail or exactness by Rosenstein, and his essay is loaded with errors. It was in the foundling hospitals of Paris at the end of the last century, in the wards of Salpétrière and Bicêtre, and in the hospitals of Vaugirard and in the Capucin convents of the Rue Saint Jacques, where pregnant women and nurses attacked with syphilis were admitted, that methodical and trustworthy observations were made (1780-1810) by Colombier, Despenières, Doublet, Mahon, Cullerier, and Bertin. Since then the history of the disease has been the history of syphilis itself.]

A full consideration of these, or even a recapitulation of the respective arguments pro and con, would far exceed the limits allotted to the present article, and we will confine ourselves simply to stating the questions which most nearly concern the practical physician, and the conclusions which the accumulated observation and experience of the profession seem to justify. The points bearing upon the general subject of hereditary syphilis which exercise an important influence upon advice or opinions of the utmost gravity as regards the happiness and well-being of the individual or family may be enumerated as follows:

1. Is syphilis in all its stages transmissible (a) to the wife or husband, (b) to the offspring? Or, in other words, is it ever proper to consent to the marriage of a person who has had syphilis? If so, under what circumstances?

2. By what means or through what channels can the disease of the parents reach the child?

3. What are the pathology and symptoms of hereditary syphilis?

4. What is the treatment--(a) prophylactic, applied to the parents, and (b) curative? We may now take these up seriatim.

No more important questions can be submitted to a medical man than those pertaining to the marriage of syphilitics. Involving as it does the welfare of many individuals, modifying or fixing the conditions or circumstances of one or more lives, his opinion should be exceptionally definite and well grounded. The responsibility of advising or consenting to the marriage of a person who has once had syphilis is undoubtedly great; the responsibility of prohibiting it is, however, no less so. Matrimony is the natural condition for the majority of people. Enforced celibacy, especially in males, brings with it not infrequently a long train of attendant evils, moral and physical. It will not do to assume that professional duty is properly discharged by telling all patients to be on the safe side and to remain single for fear of inoculating wife or offspring, unless it can be clearly shown that there is a definite and unavoidable risk in every case, which continues throughout life.[3]

[Footnote 3: "The surgeon who, on account of past syphilis, forbids marriage to an otherwise {256} eligible man must remember that he forbids it at the same time to some woman, who, possibly, if well informed as to her risks, would willingly encounter them.... Respecting a malady so common as syphilis, while it is often our duty to warn, it is also not unfrequently our duty to encourage" (Mr. Hutchinson, preface to the English translation of Fournier's Syphilis and Marriage, p. vii.)]

There are two distinct methods of arriving at an answer to the question under discussion: first, by considering the probabilities in regard to the essential nature of syphilis; and, second, by carefully weighing the clinical evidence in the matter. It seems evident that belief in any particular theory of syphilis assigning it to this or that class of disease must have an important influence in determining the opinion which is held as to its curability, or at least as to its indefinite transmissibility.

If the late symptoms, the so-called tertiary outbreaks of the disease, are held to be evidences of the presence in the system of the specific virus, which has simply remained for a long period, perhaps for many years, latent or quiescent, and which is thus again manifesting its power; if syphilis is believed, accordingly, to be a practically unlimited disease, conforming to no known law as regards its duration, corresponding to no other infectious or contagious malady in having a period of termination--more or less delayed perhaps, or more or less indefinite, but still invariably present--at which time either the particular poison or the susceptibility of the system to its influence has become exhausted;--if tertiary syphilis, in other words, is regarded as simply a continuance or recurrence of the disease, differing in no essential respect, except as to the particular tissues involved, from the same disease in its early stages, it is difficult to see how marriage can ever be conscientiously recommended to a person who has once contracted it.

It is unfortunately true that in no given instance is it safe to assure a patient of further complete immunity from the disease. In any case, however mild in its course or under whatever treatment, there is always an element of doubt as to the development of subsequent symptoms. The probability of their appearance may be reduced to a minimum, the character of the case and the thoroughness of the treatment may both seem to give assurance that a cure has been completed, and yet both patient and physician may be mortified and annoyed by an outbreak of tertiary lesions. This, I think, would be admitted by every one of large experience with the disease, and indeed furnishes the chief argument to those who deny or are sceptical as to its curability. If, then, it were impossible to predict with any sort of certainty that the contagious and highly transmissible stage of syphilis would terminate during the life of the individual, it would manifestly be unwise to permit marriage, with its risk of inoculating the innocent partner and the consequent double risk to the offspring.

If, however, syphilis is, as taught by Mr. Hutchinson,[4] one of the exanthemata, having, like them, a period of outbreak, a period of efflorescence or eruption, and a period of subsidence, and followed, like them, by certain non-contagious sequelæ, which we call the tertiary symptoms, but which are merely relapses or degenerations of parts affected during the secondary stage, it becomes evident that the risk of transmission to wife or husband or children after the lapse of a certain interval becomes greatly reduced or almost nil. No one thinks of forbidding marriage on {257} account of a previous attack of small-pox or scarlet fever or typhoid fever, even although there are unpleasant consequences which sometimes follow these diseases.

[Footnote 4: The London Lancet, Feb. 5, 1876; Reynolds's System of Medicine, Am. ed., p. 423.]

Or if another and still more plausible theory of syphilis is adopted, and we look on the tertiary period as one of contraction or obliteration of lymphatics due to long-continued irritation by the new cell-growths of the secondary stage, which from the very onset affect those vessels, our views will be materially modified. During the primary period, then, when the new cells, which either constitute the poison of syphilis or are its carriers, are accumulating at the site of original inoculation, constituting the induration of the chancre, or are slowly finding their way into the general system through the lymphatic vessels, proliferating in their walls and thickening and hardening them, or during the secondary period, when they are rapidly multiplying in all the tissues of the body, the risk of inoculation or transmission would be manifestly great. When, however, by destructive metamorphosis and degeneration, either with or without the aid of drugs, they have been eliminated from the body, the contagious element disappears with them; and although here and there throughout the body some important lymphatic trunk may have undergone irreparable injury, and may have been contracted or obliterated, permitting of the accumulation of waste products until the node or gumma or tubercle which we call a tertiary symptom makes its appearance, yet the disease has lost much of its terror, and has become dangerous only to the patient himself.[5]

[Footnote 5: Clinical Lectures on the Physiological Pathology and Treatment of Syphilis, by F. N. Otis, M.D.; Syphilis, by V. Cornil, Am. ed., 1882, pp. 17-27.]

These theories are only alluded to by way of elucidation of the statement that belief in one or the other of them has an important bearing on the relation of syphilis to marriage, and because, whichever is thought to be the most plausible, they equally lessen or altogether do away with a certain proportion of the danger formerly thought to surround the marriage of a syphilitic even after a most protracted interval. It is not necessary to accept either the one or the other implicitly. The essential point is the recognition of the fact that modern syphilographers, as a rule, regard the tertiary or late symptoms as indicative of damage done during the active period--as relapses or sequelæ, and not as fresh outbreaks, of a highly contagious and transmissible disease. Their time of appearance, their entire want of symmetry, their non-contagiousness, their non-inoculability, all favor this view, and we may now see what evidence corroborative of it may be obtained from clinical facts.

It will be necessary, in the first place, to admit that there seems to be but little doubt in the minds of most syphilographers that in rare instances syphilitic children have been born to parents who had long passed the limits of the secondary period. At least the great majority of writers upon this subject speak confidently of the exceptional occurrence of such cases, and assert that syphilis may be transmitted during any of its stages.[6] If, however, we come to look for positive evidence in this respect, we will find very little that is entirely satisfactory. Cases are reported, to be sure, in which eight, ten, twelve, or even fifteen or twenty, years after the primary sore, syphilitic patients have become the parents {258} of children who showed unmistakable indications of the disease. When we examine the history of the cases, we find usually that many important points are omitted without which it is impossible to be certain of its true character. Were both parents originally infected? If not, has a recent case of syphilis occurred in the one who at first escaped? If they were both diseased originally, has either been subsequently re-infected?--a much more frequent accident than has been commonly supposed.[7] On applying these tests to the cases in question it will be found that few if any of them are thoroughly convincing. Kassowitz's observations,[8] made, it must be remembered, upon persons with whom no mercurial treatment had been employed, seemed to show that the average limit of transmissibility was about ten years, after which time healthy children began to be born. His observations were, however, incomplete in many respects, and, like all such investigations, are of course open to the suspicion of intentional deception on the part of the patients.[9] Even these cases, however, show unequivocally, as do all which have ever been recorded, the steady diminution of the transmissive power under the influence of time alone.

[Footnote 6: Belhomme et Martin, Traité de la Syphilis, p. 413.]

[Footnote 7: Cornil, op. cit., p. 20.]

[Footnote 8: Die Vererbung der Syphilis, Wien, 1876.]

[Footnote 9: See also Hutchinson, British and Foreign Med.-Chir. Rev., Oct., 1877.]

I have said that the majority of writers seem to have no doubt of the long continuance of this transmissive power in rare cases. There are, however, a few notable exceptions. Fournier, whose immense experience and acuteness of observation entitle his opinion to the utmost consideration, says that in cases of paternal heredity the duration of the force of transmission never exceeds at the maximum three or four years.[10] In no case of the many hundreds he has observed has he known a syphilitic father to infect a child--the mother being healthy--at a later period than the one mentioned. And he is equally positive that the gradual diminution and final extinction of the syphilitic reaction of the parents upon the children is a veritable pathological law, "absolutely demonstrated."[11]

[Footnote 10: Syphilis and Marriage, Am. ed., p. 87.]

[Footnote 11: Op. cit., p. 88. Of course when both parents are diseased a somewhat longer period of activity is to be expected for the poison.]

M. Mireur, a careful and accurate observer, records[12] a striking instance in which in the history of a couple, both syphilitic and untreated, eight pregnancies occurred. The first resulted in abortion at fifth month; the second, in abortion at seventh month; the third, in a stillbirth; the fourth, a syphilitic child dying in one month; the fifth, in a syphilitic child dying in forty-five days; the sixth, seventh, and eighth, in living, healthy children. To me the most interesting fact in the whole relation is that during a portion of the time, and immediately after the last three pregnancies, which resulted in the birth of healthy children, both husband and wife manifested grave tertiary syphilitic symptoms--gummata, tubercles, ulcers, etc. This is direct evidence of the strongest kind in favor of the view that syphilis ceases to be transmissible by heredity at the end of a certain period, as we know that it ceases to be contagious or inoculable.

[Footnote 12: Essai sur l'Hérédité de la Syphilis, Thèse de Paris, 1867, p. 91.]

M. Ricord long ago,[13] and even before him Astruc and Doublet,[14] had {259} promulgated the same theory, Ricord asserting that in the tertiary stages the only effect of the disease upon the children was so to modify their organization and temperament as to expose them to developments of a scrofulous character--a view of the relation between syphilis and struma which has been so ably supported in our own day by Professor Gross.

[Footnote 13: Traité pratique des Maladies vénériennes Paris, 1838, p. 644.]

[Footnote 14: Legendre, Nouvelles Recherches sur les Syphilides, 1841 (quoted by Belhomme et Martin).]

M. Bazin also[15] denies absolutely that tertiary syphilis is any more transmissible than it is communicable in other ways, although he fails to give his reasons for this belief.

[Footnote 15: Leçons sur les Syphilides, 1859, p. 35.]

Hill and Cooper state[16] that the transmissive power continues as long as the secondary eruptions are present, but usually ceases when the tertiary stage is reached.

[Footnote 16: Syphilis and Local Contagious Disorders, London, 1881, p. 62.]

Van Buren and Keyes[17] believe that fathers with tertiary syphilis certainly, as a rule, procreate non-syphilitic children; and in speaking of the fact that when the mother has syphilis the child is generally infected, they except the later tertiary stages.

[Footnote 17: Genito-urinary Disease and Syphilis, 1874, p. 521.]

Bumstead[18] and Taylor say that without mercurial treatment the danger of transmitting the disease to offspring usually persists up to the fourth year of syphilitic contagion.

[Footnote 18: Venereal Diseases, 1879, p. 739.]

Mr. Lane says:[19] "It is certainly the rule that when the parents have fully reached the tertiary stage the children born to them are free from all signs of syphilis."

[Footnote 19: Lectures on Syphilis, London, 1881, p. 65.]

Mr. Hutchinson says:[20] "It is almost an acknowledged law that parents in the late tertiary stages do not transmit taint."

[Footnote 20: The Med. Press and Circular, Aug. 2, 1882, p. 85.]

It will be seen from the foregoing extracts, which might be greatly multiplied,[21] that there is a strong tendency on the part of many authors to limit more or less strictly the period of transmissibility of syphilis even when the disease is allowed to progress without treatment. As to the facts that it becomes milder with time, both in parents and offspring, that it ceases to be conveyed from husband to wife or vice versâ, that with each succeeding year after the termination of the secondary period the chances of escape of the product of conception increase in a rapidly augmenting ratio,--there is no difference of opinion whatever. Neither is it seriously disputed that the length of time during which the disease remains active, as well as the degree of its activity, may be markedly and beneficially influenced by the administration of mercurial treatment. Under proper medication patients who have rashly or disobediently married in the height of the secondary period have been enabled to escape the danger of transmission either to spouse or offspring--have, in fact, had children born healthy and who never subsequently manifested any symptoms of the disease.

[Footnote 21: M. Diday, Traité de la Syphilis des Nouveau-nés, Paris, 1854, p. 183; M. Bertin, Traité de la Maladie vénérienne chez les Nouveau-nés, Paris, 1870, p. 142; M. Bazin, op. cit., p. 164; M. Roger, L'Union Médicale, 1865, t. i. p. 147 (quoted by Fournier).]

I may add that my own experience seems to confirm the views which have thus been set forth. I have notes of all my cases occurring in private practice in a large city--some of them, I regret to say, among personal friends or acquaintances, some of them in our own profession--and have repeatedly {260} given permission to men to marry or to resume marital relations after three years or three years and a half of mild mercurial treatment, to which during the last six months or a year had been added iodide of potassium. In many instances healthy children have been born: in none, so far as I know, has the wife or mother been directly infected. There have been a few doubtful cases in which premature deliveries or stillbirths have occurred, but in nearly every such instance there seemed to be other and entirely competent causes for the accident; and in none of them, as I learned from the father or from the obstetrician in attendance, were the children the subjects of unmistakable syphilitic symptoms.

As to the exact time at which it is safe to permit marriage, and as to the proper treatment before and after that event, it is hardly possible in an essay like this to enter into many details. Yet so much is involved in the answer to our first question that it may not be altogether out of place here to indicate briefly the views of the writer as to general methods of treatment. This is the more proper because in every case of suspected syphilis in a new-born child, in every case of threatened or actual abortion or miscarriage in the wife of a man who has at some time in his life had syphilis, these questions will present themselves, and the answers to them will greatly influence not only the diagnosis and prognosis, but even the treatment, of such cases.

1. In the first place, then, the diagnosis of syphilis should have been assured. No venereal sore can with certainty be pronounced to be syphilitic before the occurrence of general constitutional symptoms, either the early cutaneous eruptions or at least the general glandular involvement. Treatment begun prior to these developments leaves the whole case open to the suspicion of mistaken diagnosis.[22]

[Footnote 22: "It is unsafe to predict confidently that any venereal ulcer, even a soft sore attended with suppurating bubo, will entail no further consequences. There is a strong probability that an indurated sore will prove infecting; and there is a probability, though not nearly so strong, that a soft suppurating sore will not; but exceptions to both these general rules will be met with, and there is really no absolute proof of the infecting nature of any given sore but the fact of infection itself" (Lectures on Syphilis, James S. Lane, London, 1881, p. 23).]

2. The drug which should at once be begun when the character of the case is fully recognized is mercury in one of its various forms. It may be given by the mouth, by inunction, by vaporization, by hypodermic injection, according to the preferences of the physician or patient; but, however administered, it should be given in sufficient quantity--i.e. in each case the full physiological dose of that particular patient should be employed. To ascertain this the amount used should have been gradually increased until commencing symptoms of salivation are produced, when it should be diminished about one-half.

3. The quantity which has been thus determined should be given continuously, or stopping only for the management of intercurrent complications, for at least eighteen months. If during this time new syphilitic symptoms make their appearance, the dose should be temporarily raised until they have vanished, when it should be brought down again to the original amount.

4. At the end of eighteen months or two years small doses of iodide of potassium should be added to the mercurial, and this mixed treatment should be persevered in for six months or a year longer, or should be {261} still further prolonged if during that time any evidence of syphilis is seen.

5. At the end, then, of from two to three years, if no symptom has been seen for six months or a year, treatment may be stopped and the patient kept under observation for a year; and if during that time no symptom develops he may consider himself as in all probability cured. Any course of treatment less thorough than this should be set down as insufficient to afford any reasonable presumption of further immunity from the disease.

There is evidence to prove, on the other hand, that this plan of treatment, rigidly carried out, results in the majority of cases in curing the disease, or at any rate in putting the patient in such condition that he may with safety marry and may expect to have healthy children.

To recapitulate: Syphilis after a certain period, not extending much over four years where the disease is allowed to run its own course, and probably much reduced by treatment, ceases to be a contagious disease; and at about the same time or some time after loses, in the majority of cases, its capability of being transmitted.

As there are probably exceptions to the rule that this power of transmission disappears spontaneously within any specified time, it is never safe to trust altogether to the unaided efforts of nature, but a vigorous and sufficient specific treatment must be employed.

Given, however, the lapse of a sufficient time--say from three to four years as a minimum--the history of a proper and continuous plan of treatment, and the absence for a year or more of any specific symptoms whatever, and the risks of marriage are so reduced as probably to warrant a careful physician in permitting it.[23] And conversely, of course--and this constitutes the reason for introducing the foregoing matter into a paper on hereditary syphilis--in any doubtful case where such a history can be elicited, and where all these precautions have been observed, it is improbable that any taint of syphilis has been transmitted.

[Footnote 23: This refers, of course, to an ordinary case of syphilis. If the symptoms have been unusually grave, if the deeper tissues or the viscera have been seriously involved, if cerebral or spinal complications have occurred, the situation is of course much more grave, and no step should be taken without the most thoughtful deliberation. The work of Fournier already alluded to (Syphilis and Marriage) furnishes an admirable guide under these circumstances.

Mr. Frederick Lowndes, surgeon to the Liverpool Dock Hospital (Lancet, July 8, 1882), says: "Each case must be judged on its own merits. When the constitution is good, and there has been sufficient specific treatment, marriage may be permitted within a much shorter period than M. Fournier suggests, and with safety. Syphilis alone and syphilis combined with scrofula are two very different foes to contend with, and if our patient be of a scrofulous temperament a delay even longer than M. Fournier's may be desirable."

He quotes Dr. Thomas Edward Beatty in an address at the annual meeting of the British Medical Association at Leeds in 1869: "Mercury given to the man when first diseased would, I firmly believe, have prevented this terrible calamity--i.e. the syphilitic infection of the wife; and I would now humbly suggest to all who undertake the treatment of venereal disease that if they have a certainty that their patients will remain celibate all their lives, they may heal up their sores and dispel their eruptions and sore throats in any manner they like, but that they have no right to expose the pure, innocent, high-minded females of society to contamination by marrying men treated without mercury."]

Beyond this in positiveness of assertion it is not safe to go. There may be exceptions to these as to most other hygienic or therapeutic rules, but they will surely be of excessive rarity.

{262} Before considering the methods by which syphilis can reach the child from one or the other of its parents, it may be well to mention the modes in which they can infect each other.

The father can derive syphilis from the mother only in the usual way--i.e. by contagion through a breach of surface permitting of the direct absorption of the poison, the development of the disease being attended by the usual phenomena--chancre, lymphatic enlargement, skin eruptions, etc. The woman may--and in the vast majority of cases does--acquire the disease from the husband in a similar manner. But there seems to be good reason for believing that she may also become infected through the medium of the child, who receives its syphilis directly from the father, the mother up to the time of conception having escaped contagion. More than this, it appears to be highly probable[24] that no woman ever bears a syphilitic child and remains herself absolutely free from the disease.

[Footnote 24: The strongest argument against the theory that every woman who has had a syphilitic child has herself been infected lies in the existence of cases like the following, several of which have been observed: The wife of a man having active but untreated syphilis gives birth to one or two syphilitic children, she herself developing no symptoms. Later, the husband is placed on mercurial treatment. She then conceives and gives birth to a healthy child. He stops treatment, and she again bears a syphilitic child, which on his resuming his mercurial course is followed by another healthy infant. Such cases certainly indicate that the syphilis of the mother, if any exists, is incapable of transmission to the child, as the effect of the treatment of the father is too direct and unvarying to admit of doubt.]

The existence of this form of infection--syphilis by conception--has been vehemently denied by many authors[25]--by some on the theoretical grounds that as the essential elements or carriers of the syphilitic virus have been shown to be cells or protoplasmic particles, and as, after conception, the embryo is supplied with serum, but not with cells of any kind, it is impossible that syphilis can be conveyed either to or from it;[26] by others on reported observations of numerous cases in which mothers who have been delivered of syphilitic children have shown no evidences of the disease.

[Footnote 25: Kassowitz, Von Baerensprung, Bidenkap, and others.]

[Footnote 26: Bumstead and Taylor, op. cit., p. 742.]

It would appear, however, that, setting aside arguments based on theoretical considerations, the weight of clinical evidence is altogether in favor of the frequent, if not the invariable, contamination of the mother through the medium of the foetus. No physician of large experience in this class of cases can fail to have seen some in which the husband, having had syphilis and having married after an insufficient interval or an imperfect course of treatment, has infected his wife with the disease, although at the time no discoverable symptom is to be found upon his body--no abrasion, sore, mucous patch, no lesion of continuity or suspicious point of any description. An equally careful inspection of the woman will also in such cases be attended by negative results--no initial lesion, no spot of induration, no adenopathy being at all discoverable--and yet she will be found with unmistakable evidences of constitutional syphilis.[27] There is a clue to all such cases which will immediately resolve the difficulty. In every instance, providing that no mistake has {263} been made and that both husband and wife are really free--the one from any contagious lesion, the other from any evidence of a present or previous primary sore--it will be found that pregnancy has occurred; that the woman has either been delivered of a syphilitic child or has had an abortion or miscarriage at some time before the outbreak of the symptoms of syphilis. Although I am firmly convinced that this is a statement of facts based upon careful clinical observation, and although this view has received the unqualified endorsement of no less an authority than Fournier,[28] it is yet strongly combated by many excellent authorities. They say in reply to the above arguments that the reported cases are open to just criticism, that trifling and unnoticed lesions of the father--chafes or abrasions almost microscopic--suffice to transmit it on his part; while as an explanation of the supposed absence of the primary lesion in the mother they plead the well-known difficulty of discovering it in women under any circumstances.

[Footnote 27: For a typical case see Colles's Works, New Sydenham Society, London, 1881, p. 253. From that date to this hundreds of such cases have been observed, and it would be idle to refer to them.]

[Footnote 28: Op. cit., pp. 26-30. He confesses to complete ignorance as to the precise method by which this contagion takes place, whether by poisoning of the fecundated ovule at the moment of conception (the theory of Von Baerensprung) or by exchanges of the utero-placental circulation, and regards the various hypotheses upon these points as without practical value.]

Another argument, however, which seems to me to be unanswerable lies in the application to the case in question of the well-known "law of Colles," which from the date of its first enunciation by its distinguished expounder in 1837 down to the present day has been found to be absolutely without exception. I know of no other statement in reference to disease which is at once so sweeping and comprehensive in its bearings and so completely substantiated by clinical experience. It may be given in his own words: "One fact well deserving our attention is this: that a child born of a mother who is without obvious venereal symptoms, and which, without being exposed to any infection subsequent to its birth, shows this disease when a few weeks old,--this child will infect the most healthy nurse, whether she suckle it or merely handle and dress it; and yet this child is never known to infect its own mother, even though she suckle it while it has venereal ulcers of the lips and tongue."[29]

[Footnote 29: The Works of Abraham Colles, edited by Robert McDonnell, the New Sydenham Society, London, 1881, chap. xiii. p. 287.]

As to the absolute and unvarying truth of this law there is no excuse for a shadow of doubt. To quote Mr. Hutchinson: "It has received the assent of every authority who has written on the subject since it was announced. It has attracted attention both at home and abroad, and I am not aware that a single exception to it has been recorded.[30] We have all of us seen chancres on the nipples of wet-nurses. They are, indeed, not very infrequent. We have, however, none of us seen such on those of the mothers of infected children. Let us remember that it is very unusual to put a syphilitic infant out to wet-nurse--a thing which no prudent physician would ever permit--and that, probably, for one so nursed a hundred are suckled by their mothers, and we can appreciate the weight which this entire absence of proof that mothers ever suffer {264} bears. It amounts, I think, to all but proof that they are absolutely insusceptible."[31]

[Footnote 30: The cases of Cazenave (1847), Cocchi (1858), Müller (1861), Ranke (1878), Guibout (1879), Scarenzio (1880), and Zingalès (1882), are defective in important particulars, the first two so much so as to render them valueless. Ranke's case is by far the strongest yet recorded, but lacks fulness and rests upon his unsupported testimony. (See Hill and Cooper, op. cit., p. 55, and Nouveau Dict. de Méd. et de Chir., vol. xxxiv. p. 687.)]

[Footnote 31: Dr. J. N. Hyde suggests (Archives of Dermatology, April, 1878, p. 103) that "the full weight of Colles's law is to be estimated in connection with the question whether the child whose hereditary syphilis is derived from the mother exclusively is capable of infecting its healthy father; and if no instance of this latter can be adduced a higher law becomes defined--viz. that the child whose hereditary syphilis is transmitted by one parent only is incapable of infecting either." He explains this hypothetical immunity, in which he seems to believe, by saying that "it is probably due to the fact that the syphilis-bearing cell-element cannot readily be implanted upon the soil from which it sprang--a fact illustrated by the infecundity of consanguineous marriages and the non-auto-inoculability in general of the primary lesion of syphilis." When, however, we exclude the large number of cases in which the father is already syphilitic, and remember that in the others the contact between him and the child is slight, infrequent, and fleeting--being usually limited to an occasional kiss or caress--it does not seem strange that no instances of such infection have been reported; nor does the fact seem at all worthy of being made the foundation of a general law.]

There can be but one rational explanation of these facts--viz. that the mothers who have thus acquired an immunity have done so by first acquiring the disease. It may, in those cases where no secondary symptoms appear, be in a modified form, due, as suggested by Mr. Hutchinson, to some heteromorphism or alternation of generations on the part of a specific fungus, or to the excessively small quantity of the poison which finds its way from child to mother, or to some other obscure cause with which we need not now concern ourselves. It is, at any rate, no more mysterious than the protective influence of vaccine in small-pox, and when one is rationally explained doubtless the other will be found to be closely allied in its mode of action. The fact which interests us at present is that it is in the highest degree improbable that anything but some form of syphilis itself could afford this entire protection, and that it is not in the least unreasonable, but, on the contrary, logical and consistent with all the known facts, to suppose that while in some cases no observable symptoms might be produced, in others where the cause was the same, but more active, or the powers of resistance less, the usual constitutional phenomena would be developed.

We may conclude, then, that the husband may infect his wife--(1) In the usual manner or by direct contagion; (2) through the medium of the child, or at any rate by the production of conception.

The theory upheld by Von Baerensprung, that the syphilis of the mother is imparted to her at the moment of impregnation, the disease being impressed upon the fecundated ovule, does not materially conflict with the above views, conception being in either case the essential factor, but in the latter instance the intervention of the foetus itself not being necessary. It has to support it the clinical fact that in those cases where syphilis appears during pregnancy the outbreak of symptoms occurs at about the ninth or tenth week after the date of conception, or a period which closely corresponds to that of the appearance of general symptoms after exposure to ordinary contagion--allowing about three weeks for the so-called incubation of the chancre and six weeks for the secondary incubation.[32]

[Footnote 32: This has been shown not only by Von Baerensprung (Die Hereditäre Syphilis), but also by Diday, whose observations were intended to prove the possibility of syphilis being derived from the child by the mother--"choc en retour." In 24 cases the period at which the first eruption appeared in the mother averaged sixty-five days after conception; only once did the first signs appear after the fourth month of pregnancy.]

{265} It may also be said to be rendered probable by the following line of argument:

Colles's law, which is without exception, demonstrates that every woman who has had a syphilitic child has been herself infected, even if she has had no observable symptoms;[33]

Cases are recorded, however, in which a woman having given birth to one or more syphilitic children, and therefore herself syphilitic, bears healthy ones in consequence of specific treatment administered to the father before and during the period of conception, she remaining untreated;[34]

The determining cause, therefore, of the syphilis of the child is not the syphilis of the mother, but the condition of the fecundating germ of the father; and, as a corollary,

The determining cause of the syphilis of mothers in whom the disease follows conception is not by infection from the foetus through the utero-placental circulation[35] or otherwise, but is the diseased male procreative cell which becomes blended with the female ovule.[36]

[Footnote 33: It is obviously no explanation of the law of Colles to say that "it would seem to indicate that the escape of the mother is due to some occult, undiscernible change in her system" (Bumstead and Taylor, op. cit., p. 745).]

[Footnote 34: See foot-note, p. 262.]

[Footnote 35: On account of the absence of cellular elements in the fluid interchanged.]

[Footnote 36: An elaborate paper by Fraenkel (Archiv für Gynaekologie, 1873, vol. v. p. 1), based on twenty-one cases of childbirth, was written to prove by the condition of the placenta that direct infection of the child by the father was possible without the participation of the mother, and that when the latter became infected it was through the medium of the child (choc en retour). In fourteen of his cases the specific change in the placenta began in the foetal portion or affected it exclusively. These cases, however, as analyzed by Hill and Cooper (op. cit., p. 57), are altogether defective in important particulars. Of course to sustain his theory there should be indubitable evidence that the fathers were syphilitic, and that the mothers were not so. So far from this being the case, but one of the fathers was known to be syphilitic, while two of the mothers had evidences of constitutional syphilis at the date of childbirth, and nine others had markedly suspicious histories. The lesions of the placenta which are thought to be syphilitic consist in the development of papular or gummatous growths which give rise secondarily to inflammatory troubles affecting either the placenta itself or the placenta and the uterine mucous membrane.]

If the premises are admitted the conclusions seem irresistibly to follow.

There is no proof whatever that the semen of a syphilitic man is contagious or can transmit the disease in any but the way above discussed. On the contrary, it has been shown experimentally[37] that it is entirely innocuous and non-inoculable.

[Footnote 37: Mireur, Annales de Derm. et Syph., 1876, p. 77.]

All other theories as to methods of contagion are so entirely hypothetical and unsupported by trustworthy evidence that we can afford to disregard them.

We may now consider the ways by which syphilis reaches the child, and they may be broadly classified into--

1. By descent from the father.

2. By descent from the mother.

3. By direct infection.

As a matter of course, the influence of the father upon the child, so far as regards heredity, ceases at the moment of conception; or, to be more exact, no subsequent condition of the male parent, no development or acquirement of disease, can exert any further effect. That the existence of active syphilis in the father may result in the transmission of the malady to the child can hardly be doubted. To be sure, there are numerous {266} examples of cases where both wife and child have escaped though the husband showed at the time of conception very active secondary lesions. These, however, are exceptions, and do not in the least invalidate the rule that it is possible for a father to hand the disease directly to his child. There is no other possible interpretation of the cases already alluded to in which successive pregnancies in the same woman alternately resulted in healthy or in syphilitic children according as the father was or was not kept under specific treatment. Other arguments might be advanced, but that seems to me conclusive.

The relative effect of paternal as compared with maternal influence may be considered after we have described the latter.

Descent from the mother may occur theoretically in consequence of--

1. Infection of the mother previous to conception.

2. Infection of the mother at the moment of conception.

3. Infection of the mother during the period of utero-gestation.

As to the first of these methods of transmitting the disease there is little if any difference of opinion. Even those who claim the most for paternal influence[38] include among the conditions which may give rise to syphilis in the child disease of the ovule, and it may be stated as incontrovertible that recent or active syphilis in the mother at the time of conception will almost certainly be followed by syphilis in the child. As a rule, women who have borne syphilitic children, even when they do not give unmistakable evidence of the disease, fail in health, become anæmic, and often develop glandular or osseous swellings which, according to Zeissl,[39] are only relieved by antisyphilitic treatment. The bearing of Colles's law upon the alleged immunity in many instances of the mothers of syphilitic children has already been shown (p. 264); and there is other evidence, not perhaps so conclusive, but strongly corroborative, of the same view--viz. that their escape is only apparent, and that syphilis, either latent or active, always affects such mothers.[40] There can be no manner of doubt that in every instance at or about the time of childbirth there are not to be found pathognomonic lesions of syphilis, nor do such lesions always make their appearance in cases where the period of observation is a short one; but it is claimed with much show of truth that prolonged and patient inspection of such patients will in time result in the discovery of some symptom which betrays the presence of the disease.

[Footnote 38: Bumstead and Taylor, op. cit., p. 745. Hutchinson in Reynolds's System of Medicine, Am. ed., vol. i. p. 431: "In a large proportion of the cases met with in practice the taint is derived from the father only." (On this point see foot-note to p. 270.) If there were any doubt as to the fact that syphilis in the mother only may be transmitted to the child, it would be removed by the cases of Bardicet and others mentioned in Nouv. Dict. de Méd. et Chir., vol. xxxiv. p. 688. In these cases nurses who had contracted the disease from their sucklings subsequently became pregnant (without having infected their husbands) and gave birth to syphilitic children.]

[Footnote 39: Jahrbuch, vol. ii. p. 303, 1872.]

[Footnote 40: Zeissl, Sigmund, Oewre, Flindt, Woodman, and others are quoted by Hill and Cooper to this effect. Woodman, for other purposes, gave the histories of 200 cases of infantile syphilis. In all of these the mothers had suffered from typical secondary lesions.]

The cases in which treatment of the father has resulted in healthy children, whereas without treatment he procreated only syphilitic children, the mother being without either symptom or treatment, have been urged as evidence of the direct descent of syphilis from the father to the child without the intervention or participation of the mother. Doubt {267} has been thrown upon them by those who uphold the contrary view;[41] but after looking into them carefully I am constrained to admit that some of them, notably those of Kassowitz[42] and R. W. Taylor,[43] are convincing of the facts--(1) that treatment of the father controlled the condition of the child, and (2) that there was no evidence that the mother had syphilis. But we have seen that the only proof of the universal infection of mothers of syphilitic children is the law of Colles, and that in many cases the disease is for a long time latent or unrecognizable. Let us admit that this was the condition in the cases in question; it does not at all follow, necessarily, that because the mother has latent or hidden syphilis she must infect her child. Every case even of active syphilis in the parents is not handed down to the children, a certain proportion of whom escape even when both parents are in the height of the secondary stage at the time of conception.[44] But the activity of the disease in the children, and even more the likelihood of its reaching them, are in direct proportion to its activity in the parents.[45] A mother, therefore, who under the influence of active syphilis in her husband has given birth to two or three syphilitic children, and has herself shown no symptoms of the disease, may nevertheless have it in the latent form and have no strong tendency to transmit it. Consequently, treatment of the father will result in the procreation of healthy children, because it removes the active and efficient cause of their infection. To take any other view of these cases is to assume that every syphilitic parent must hand down the disease to the children--an assumption which is not in consonance with numerous well-attested clinical facts.

[Footnote 41: Hill and Cooper, op. cit., pp. 52, 53.]

[Footnote 42: "Die Vererbung der Syphilis," Stricker's Med. Jahrb., 1875, p. 391.]

[Footnote 43: Archives of Clinical Surgery, New York, Sept., 1876.]

[Footnote 44: Fournier, op. cit., pp. 35-37.]

[Footnote 45: Hutchinson, op. cit., p. 431.]

Leaving this interesting question, however, we may consider the other methods by which syphilis descends from the mother to the child, having seen already that it is beyond doubt that it may be handed down by disease of the ovule due to syphilis acquired previous to conception, and having seen that there is a high degree of probability that the mother herself rarely, if ever, escapes the disease. The second method, or that in which the mother becomes syphilitic at the moment of conception, has already been sufficiently discussed. It is really, strictly speaking, an example of paternal heredity, as the resulting germ is syphilitic--not because the ovule of the mother was infected, but on account of the disease of the spermatozoid of the father.

There remains for consideration the influence upon the child of a syphilis acquired by the mother during some period of utero-gestation. That under these circumstances the child can become infected has been and is still absolutely denied by some very respectable authorities.[46] All that is {268} necessary for proof of its occurrence is, however, (1) freedom of both parents from syphilis at the time of conception, or, in other words, syphilis must have been acquired by both--not alone by the mother--after the beginning of pregnancy; (2) that the syphilis of the child be unmistakably pre-natal--that is, not acquired by some accident during or after birth.

[Footnote 46: Bumstead and Taylor, op. cit., pp. 742, 744. They base their denial, first, on the physiological fact (?) that no interchange of cellular elements between mother and father is possible, and next on the absence of satisfactory evidence of the occurrence of infection during pregnancy. Zeissl's case seems sufficient answer to the last assertion, and there is strong evidence that the first is without good foundation. "The placenta is penetrated by the virus, and does not play the part of a filter for the elementary particles of matter which, so far as we know, represent the true active contagion of the disease. In the absence of direct experiments, which it would be almost impossible to institute, we may argue from the facts known to exist in certain acute infectious diseases in which there are very interesting points of resemblance.

"It has been known for a long time that small-pox occurring in the mother may be transmitted to the product of conception enclosed in the uterus, and it is supposed that the virulent particles traverse the walls of the maternal vessels in order to penetrate the circulating apparatus of the foetus. But what is only a supposition in the case of small-pox seems to have been actually demonstrated in symptomatic charbon (Arloing, Cornevin, and Thomas), bacteridian charbon, and recurrent fever, in which the poisonous element is easily recognized. The recent experiences of Strauss and Chamberland (1882) have shown that the foetus participates in the 'infection charbonneux' of the mother. Albrecht has shown (1880) the presence of numerous spirochoetæ in the blood of the heart of a child born at seven months of a woman with a second attack of relapsing fever. It may be supposed, therefore, though not demonstrated, that the transmission of syphilis takes place by the same method as that of relapsing typhus or of charbon" (Nouveau Dict. de Méd. et de Chir., pp. 682, 683).]

The following case,[47] reported by a most accurate observer, seems to combine both these requisites. Zeissl the younger reports that O. X., thirty-six years old, never having had syphilis, left his wife, to whom he had been married two years, to go a journey on July 15, 1877. The wife was then in the second month of her first pregnancy. On July 24th O. X. had extra-marital intercourse. About twenty-one days after this coitus he observed a small lump on the inner surface of the foreskin, and on Aug. 22d he consulted Zeissl the elder. On Sept. 23d a maculo-papular eruption of the skin with erythema faucium appeared. Under treatment these symptoms completely disappeared. On Oct. 29th he went home to fetch his wife to Vienna for her lying-in, and had intercourse with her soon after his return, notwithstanding Zeissl's strict prohibition. At the beginning of December a hard sore developed on the left nympha of the wife, who was then in the seventh calendar month of her pregnancy. At the end of December a maculo-papular eruption spread over the body and was treated with mercury. On Feb. 14, 1878, a well-grown and apparently healthy female child was born at full term. When eleven days old[48] a pustulo-scaly eruption came out on the child's soles and toes, and soon afterward a maculo-papular eruption over the body generally. A few days later the child died. No post-mortem examination was permitted. In July, 1878, the wife had iritis, and after that gummata on the leg. She miscarried in July, 1878, at the third month, and again in February, 1879, at the second month.

[Footnote 47: Quoted by Hill and Cooper, op. cit., p. 60.]

[Footnote 48: Of course much too early for constitutional symptoms if the disease had been acquired during or after birth.]

There seems to be no reasonable escape, after reading this carefully, from the conclusion that in some manner the poison of syphilis found its way from the mother to the child. The old idea that the latter was directly infected in utero from the semen of the father is altogether without foundation. Other cases equally satisfactory and complete have been reported, and, unless the intelligence or the truthfulness of the observers be impugned, establish without doubt the possibility of infection during utero-gestation.

In the above case the contagion of the mother occurred in the seventh {269} month of pregnancy; and this, I believe, is as late as it has ever been known to be communicated to the child. The exact date at which it becomes impossible so to transmit it is unknown, but as a general rule it may be said that the earlier a mother is infected during gestation the less likely is it that the child will escape. Treatment of the mother--as of the father in cases where he is at fault--very greatly modifies the whole problem and adds immensely to the chances that the child will not be infected.

Direct infection of the child during birth could not properly come under the head of hereditary syphilis. There is no possible reason why, when the mother has contagious lesions of the genitals, acquired too late to infect the child in utero, this should not occur, but as a matter of fact no such case has ever been recorded. One explanation of this circumstance may be found in the protective covering of vernix and mucus which coats the infant's body and lessens greatly the risk of absorption. This hardly accounts satisfactorily, however, for the entire absence of such cases from medical literature, and it is fair to suppose that in all but those cases in which the primary sore is acquired during the last month of gestation--which for obvious reasons are excessively rare--the infant acquires some immunity which protects it from its mother, and is similar to that which, under Colles's law, operates in her favor. In other words, even though apparently free from syphilis at birth--a not uncommon event, as we shall see--it has a latent or modified syphilis which protects it from contagion.

We may now briefly restate the conclusions at which we have thus far arrived:

1. After a certain interval, not less than four years, and after thorough specific treatment, a person who has contracted a syphilis not especially severe or malignant in its type may be permitted to marry. The assent to marriage will then be based on a belief in the curability of syphilis or the cessation of its contagiousness, its inoculability, and, in the vast majority of cases, its transmissive power at the end of the secondary stage.

2. It may be inherited from either parent or from both, and the probability that this will occur increases in a direct ratio with the nearness of the time of conception to the date of their infection with the disease. The severity of the inherited disease in the child increases in the same proportion.

3. It is undoubted that, the father being healthy and the mother syphilitic, the child may, and in all probability will, have the disease.[49]

[Footnote 49: Dr. Sturgis, who disbelieves altogether in the possibility of paternal heredity, concludes, after examining the subject carefully, that (1) a mother begets non-syphilitic children as long as she is not infected, even though the father is syphilitic; and (2) the moment she is diseased the children are inevitably so (Paper on "The Etiology of Hereditary Syphilis," New York Medical Journal, July, 1871). This doctrine was previously supported by M. Cullerier, whose views gave rise to the remarks of M. Voillemier (quoted by Fournier) that if they were accepted "the father would be only the accidental occasion of a child; one would be, in reality, the child of his mother only." Cullerier's cases are invalidated by the fact that the syphilitic fathers who had healthy children had been subjected to mercurial treatment (Mém. de la Société de Chirurgie, Paris, 1854, quoted by Taylor in Archives of Clin. Surg., vol. i. p. 83). The theory is a very old one. Vassal has sustained this idea as long ago as the end of the last century. Kostum (1804), and after him Hufeland, were of the same opinion. Cullerier (1857) wrote: "In order that a child acquire syphilis hereditarily it is necessary that the mother is or has been {270} syphilitic." Notta, Follin, Charrier, Mireur (1867), and Langlebert (1873) support this theory more or less earnestly. Oewre wrote (1873): "Paternal influence is nil as regards hereditary syphilis." Isseff (1879) wrote: "Where a man suffers or has suffered from syphilis he cannot transmit the disease to his descendants without infecting his wife; that is to say, in fewer words, there is no infection from the father." Sigmund says: "The heredity of syphilis is derived in its last analysis from the mother" (Nouveau Dict. de Méd. et Chir., vol. xxxiv. p. 689).]

4. It is probable, but less so,[50] that, the mother being healthy and the father syphilitic, the child will be infected.

[Footnote 50: This refers simply to the comparative probability of infection, and does not conflict with the statistical fact expressed by Hutchinson (Reynolds's System of Medicine, vol. i. p. 431) in his words: "In the large proportion of cases met with in practice the taint is derived from the father only." This numerical predominance of paternal influence is very readily explained. There are many more syphilitic men than syphilitic women, and especially among the couples who contract fertile marriages the number of women who are infected before becoming mothers is inconsiderable. On the other hand, it frequently happens that men who have had syphilis, but have been without symptoms for a longer or shorter interval, marry and transmit to a series of children a disease which has ceased to be directly contagious to their wives, the transmissive power continuing after the possibility of ordinary contagion has disappeared. As in the majority of such women the disease is latent, and may be only displayed in their immunity from infection, it becomes evident that, history and symptoms both being wanting on their part, the conditions justify the assertion of Mr. Hutchinson. (See Nouveau Dict. de Médecine et Chirurgie, p. 684.)

That assertion (quoted above) has, however, been thought by several writers to indicate his belief in the escape of the mother. That I have not misinterpreted him is evident from the following extract from an article on "The Transmission of Syphilis," written by him (Brit. and For. Med.-Chir. Rev., Oct., 1877): "I take it for granted (although I know that there are still some who doubt) that it is possible for a father to transmit the taint, the mother being at the time of conception wholly free. I believe, indeed, that in practice this is by far the most common way in which syphilis is transmitted. Whether in these cases it is correct to speak of the inheritance being paternal only is, as we have just seen, another matter, since it is possible that in every instance the mother derives an infection from the father, and may thus in turn influence it."]

5. It is highly probable, though it can hardly be considered as proven, that in all cases where a child becomes syphilitic through paternal influence the mother is also the subject of syphilis, which may, however, assume a latent form, the only evidence of its presence in a few cases being the protection which it affords against contagion through the medium of the child.

6. Syphilis may be transmitted from mother to child even when it is acquired by the former as late as the seventh month of utero-gestation.

Since writing the above the thirty-fourth volume of the Nouveau Dictionnaire de Médecine et de Chirurgie has been published. In the article on syphilis seventeen pages are devoted to the question of heredity, which is reviewed in a most thorough manner and finally summed up as follows (p. 698):

"The most definite views which we possess on the subject of the hereditary transmission of syphilis may be thus expressed:

"Children may be infected by heredity, not only when the two parents are syphilitic, but also when only one, either the father or mother, is diseased at the time of conception.

"When both parents are diseased at that time there is more certainty that the child will be infected, and infected gravely, than if only one of them has the pox.

"The hereditary disease is not always fatal, even when both progenitors have actual specific symptoms. The more recent the disease of the parents the greater the chances of their transmitting the disease and of its assuming {271} a serious form. There is no proof that inherited syphilis is more grave when derived from the father than when coming from the mother.

"It is altogether exceptional for the mother, healthy at the moment of conception, not to participate in the disease transmitted by the father to the child. If she escapes direct contagion--which is rare when the disease of the father is active--she undergoes a species of infection from contact with the contaminated foetus.

"Syphilis by conception, which is thus transmitted from the foetus to the mother, may present the usual characters and evolutions of the acquired disease; frequently, also, it is latent, and is betrayed only by the existence of immunity from further contagion on the part of the mother. It may finally manifest itself by tertiary symptoms or by systemic troubles without specific characters.[51]

"The power of transmitting syphilis hereditarily decreases spontaneously as the disease of the parent becomes older. The influence of treatment is no less certain than that of time. When the two progenitors are at the moment of conception free from syphilis, the foetus may still be infected if the mother acquire the disease during her pregnancy."

[Footnote 51: "This form of syphilis shows itself in the mother in three ways:

"1st. By the usual signs of syphilis by contagion, with the exception of the primary sore, appearing about the sixty-fifth day after conception.

"2d. Appearing at a later date as secondary or even as tertiary symptoms, and preceded merely by a little disturbance of the general health, unhealthy appearance of skin, falling of hair, etc., but nothing truly specific.

"3d. Showing itself some years afterward in a tertiary form, having in the mean while given no indication of its existence save only in the protection it afforded against contagion from the child" (Nouveau Dict. de Méd. et Chir., vol. xxxiv. p. 696).]

* * * * *

Syphilis of the placenta is of especial interest in its relation to the abortions and stillbirths so frequent in syphilis. Until the elaborate paper of Fraenkel upon this subject (see foot-note, p. 265) almost nothing was known about it. He describes[52] the macroscopic changes as consisting of increased size and weight of the placenta, closer and firmer texture of the placental tissue, the presence of old and recent extravasations of blood in all stages, opacity and thickening of the decidual covering and of the amnion and chorion, which are in places adherent to each other. Microscopically, it was found that the placental villi were filled with small nucleated cells, which were especially abundant in the centre of the villous spaces along the axis where the vessels usually take their course. The ends of the villi were enlarged with knob-like processes.

[Footnote 52: I condense here from the translation of Fraenkel's paper, which constitutes the bulk of chap. xxvii. of Bumstead and Taylor's excellent work.]

Fraenkel's explanation of these changes is as follows: Under the influence of syphilis[53] cell-proliferation begins in the villi, which are, normally, only sparingly supplied with cells. These new cells excite proliferation of the cells of the connective-tissue stroma and of the epithelium. This proceeds to such an extent that it leads to compression of the vessels,[54] interfering with the circulation, and finally obliterates them. The {272} vascular spaces into which the villi dip become filled up and narrowed, and often disappear. In this way, and also by reason of the thickening of the epithelium, the interchange between the maternal and foetal blood is interfered with, and at last is prevented.

[Footnote 53: Fraenkel took as his criterion of syphilis the presence of the osteo-chondritis described by Wegner (see p. 286).]

[Footnote 54: "Hennig was the first who called attention to the intimate relation of the cell-growths to the vessels. The obliteration or compression of numerous vessels of the villi interferes with the mutual interchange of gases between the maternal and foetal blood, causing fatty degeneration of the villi and, if the process is extensive, the death of the foetus" (article "Syphilis," Ziemssen's Cyclopædia, vol. iii. p. 237).]

If this process is spread over the whole placenta, the foetus perishes before it is complete. If it is limited to circumscribed areas, it may continue to live for a shorter or longer period.

These observations require to be repeated and confirmed, but they have served to open up a most interesting branch of syphilitic pathology.

Syphilis in the parents will manifest itself in the children in one of several ways, which are determined chiefly by two factors--viz. first, the length of the interval between the infection of the parent and the date of conception; and, second, the thoroughness of the treatment of the parents during that interval. To these may be added as subsidiary, but still of definite importance, a third, the type of disease which has affected the father or mother, whether mild or severe, benign or malignant.

From what has already been said in reference to the question of marriage, it will be at once understood that the danger to the offspring in untreated cases, and in those where conception has occurred during the early secondary period of the disease, is of the most extreme gravity.[55] In such cases the usual result of pregnancy is abortion at from the first to the fifth or sixth month,[56] the foetus sometimes exhibiting the evidences of the disease in the shape of large bullæ upon the palms and soles, or in the presence of characteristic visceral lesions, but quite as often showing nothing distinctive. It has generally undergone more or less maceration, and the skin, which is readily detachable, is of a congested, purplish color.

[Footnote 55: According to Kassowitz, in women who are not treated all pregnancies occurring within the first three years of their infection terminate either in abortion or in the birth of children who survive for only a few weeks or months.

Weber (quoted by Parrot, Le Progrès Médicale, Nov. 24, 1877, p. 882) treated thirty-five pregnant women by mercurial inunction, and they all went to full term under normal conditions. Among those treated with mercury and iodide of potassium, but who, by reason of intolerance of the former drug, took chiefly the latter, 20 per cent. aborted; when the mixed treatment was carried out regularly 15 per cent. aborted; and when only iodide of potassium was given 36 per cent. aborted. His studies were made on 109 syphilitic pregnant women. Parrot himself says (ibid.) that "abortion occurs in about one-third of all syphilitic women. Syphilis should always be suspected when this accident occurs several times successively." "The date of abortion depends on the age of the syphilis of the woman. It is most to be feared during the evolution of secondary symptoms, particularly when they are grave. There is more probability that it will occur when infection has preceded pregnancy than when it occurs during its course. Nothing is so much to be feared as contamination of the ovule. The nearer the date of infection of the mother approaches to full term, the fewer the chances of abortion. It is not likely to occur even when the mother is infected at five months."]

[Footnote 56: Many observers think that abortion results directly from the death of the foetus. Babington (notes to Hunter's Treatise on Venereal), Trousseau, and Von Baerensprung were of this opinion.]

Dr. Cory thinks that in many cases it is possible that the effect of syphilis may be to effect so early an abortion that the case is simply regarded as one of delayed menstruation or of menorrhagia. Such a conception would, however, be competent to infect the mother, and might seem to explain cases otherwise involved in obscurity.[57] At least one-third {273} of all syphilitic children are dead born.[58] As time goes on, however, and the intensity of the parental disease is lessened, or in cases where appropriate treatment has been applied, either the abortion occurs at a later period of pregnancy or the children are brought alive into the world. Even then, however, and although at birth they may show no evidences of the disease, their chance of escape is but small. One-fourth of them die within the first six months. If they survive that period, the chances for life are slightly in their favor, but those for health or freedom from deformity and disease are still overwhelmingly against them.

[Footnote 57: As, for instance, when a woman married to a syphilitic man, but without issue, remarries a man with no history of syphilis, and yet gives birth to a syphilitic child. In such a case there would be no history of direct infection and none of pregnancy, the only two modes by which she could have contracted the disease, and the father--the second husband--might be unjustly suspected.]

[Footnote 58: Kassowitz, op. cit.]

The course of inherited syphilis differs strikingly from that of the acquired disease. It will hardly be necessary to do more than remind the reader of the ordinary stages of the latter affection--the primary, which includes the period of the chancre and of lymphatic engorgement, lasting about sixty to seventy days; the secondary, or exanthematic, characterized by copious cutaneous eruptions and extensive involvement of mucous surfaces, lasting from one to three years; the intermediate,[59] or the stage of latency and relapses, lasting for a very variable period, from three to ten years, but under proper treatment very much reduced or altogether abolished; and finally, the tertiary period, beginning four or five years after contagion, extending indefinitely throughout life, but often in cases properly treated absent altogether.

[Footnote 59: So designated by Mr. Hutchinson, and for clinical purposes a very valuable addition to the periods of syphilis. He describes it as follows: The patient may be either wholly free from symptoms and in good health, or he may remain pale and rather feeble, and liable from time to time to slight returns of eruption on the skin, sores on the mucous membranes, condylomata, etc. He is protected as regards fresh contagion, and should he beget children they are almost certain to suffer. The relapses during this stage are usually easy to be distinguished from true secondary symptoms. There is little or no febrile disturbance, the rash is not copious, and often not symmetrical. Acute iritis, retinitis, etc. never occur for the first time, though they may do so in the form of relapses.]

For purposes of description and of contrast we may similarly divide the whole period of evolution of a case of inherited syphilis,[60] omitting the primary stage, which has never been found to exist in true cases of hereditary syphilis. Of course in congenital or infantile syphilis, in which by direct contagion, either from the mother or from any one else, the disease was acquired by the child, the course would not differ materially from that observed in the adult. But as this stage in all probability corresponds to the period during which the poison is already finding its way into the system through the lymphatics, of course it is not found in the child who is infected from the moment of conception or who receives the poison from the mother directly into the circulation.[61] For from one to three weeks the infants often show no symptoms of the disease. In 158 cases collected by Diday, 86 manifested symptoms of the disease before the expiration of the first month, and 60 of the remainder before the end of the third month.[62] When to these are added the {274} statistics of Roger, we find that of a total of 172 cases, 159 showed syphilitic symptoms before the end of the third month.[63] When the symptoms are present at birth, they consist largely in a general withered, atrophied, weazened appearance of the child; a hoarse cry, due to swelling, with subacute inflammation, or even ulceration, of the laryngeal mucous membrane; a coryza,[64] due to a similar condition of the Schneiderian membrane; and certain cutaneous eruptions, the most common of which at this early date is the large vesicular or bullous eruption known as syphilitic pemphigus.

[Footnote 60: The idea that the character of the symptoms which first appeared depended upon and corresponded with the stage of the disease in the parent has now, I believe, no supporters. It was once thought, at least by some syphilographers, that if the parents were in the tertiary stage at the time of conception the child would develop tertiary symptoms, omitting the other stages.]

[Footnote 61: If chancre were the first symptom of constitutional syphilis, why should it not appear in cases of hereditary syphilis?]

[Footnote 62: Infantile Syphilis, p. 101]

[Footnote 63: About 1823, from 16,000 to 17,000 children were admitted annually to the wards devoted to foundlings at Lyons. Clièt, recording his experience with this disease, says that syphilis is one of the most common of their maladies, but that "it exceedingly seldom shows itself at birth by evident signs" (Compte-Rendu Méd. Chir. des Observations à l'Hôpital général de la Charité de Lyon, 1823). Cristoferi, physician to the foundling hospital at Bologna, says that syphilis generally manifests itself between the ages of one and three months. Never once, he states, was a newly-born infant admitted with the disease unquestionably developed (Gazette Medica di Milano, 1844). Trousseau says that it "rarely appears before the second week, and very exceptionally after the eighth month" (Lectures on Clin. Med., vol. iv. p. 331, London, 1871, ed. of New Sydenham Society).]

[Footnote 64: Usually appears later, but exceptionally at birth.]

Pemphigus.--With regard to the specific or non-specific character of this eruption there has been much difference of opinion, and, as it is often the earliest distinctive expression of syphilis, a diagnosis of which could hardly be founded on the general appearance of the child, or even on the hoarse cry and the coryza, it becomes important to have definite ideas upon the subject. Nearly a century ago (in 1794) it was denied[65] that this eruption was a manifestation of venereal disease; and this view has been supported by many able and accurate observers down to the present day. In 1851 a discussion upon the subject took place in the French Academy of Medicine, which elicited the opinions of the majority of those members who were entitled to speak with authority in the matter, and which has since been referred to by most writers. Cazeaux upheld the non-syphilitic hypothesis on the ground (1st) that the so-called syphilitic pemphigus of children does not differ from the simple pemphigus of adults, presenting none of the physical characters which distinguish the specific cutaneous eruptions; (2d) that it appears at birth or immediately after, while the symptoms of hereditary syphilis generally show themselves later;[66] and (3d) that at that time there had been seen no cases of pemphigus at the Lourcine Hospital, where so many syphilitic children were born.[67] In this view he was supported by Trousseau, Lasègue, Gibert, Bazin, and other obstetricians and syphilographers of note.[68]

[Footnote 65: Ariander, Mémoires de Méd. et d'Accouch., quoted by Diday.]

[Footnote 66: This is now known to be an unreliable distinction, expressing perhaps a general rule, but one with so many exceptions as to render it void of diagnostic significance.]

[Footnote 67: This may have been true at that time, but has certainly not continued to be so. Cornil says: "We often see at the Lourcine children born prematurely or at the full term with pemphigus, either fully developed at the moment of birth or appearing a few days afterward, and who commonly die with syphilitic cachexia, the sad heritage derived from their maternal parents."]

[Footnote 68: Pemphigus may indeed be a specific affection, but no characteristic sign has been discovered sufficient to distinguish it from the ordinary form of pemphigus. "On the other hand, there is no inconsistency in admitting that syphilis, which so deeply impairs the constitution of the parent, may act like any other common cause and excite non-specific pemphigus; for an infant is badly lodged and poorly nourished in the womb of an enfeebled mother, apart from the influence of the virus" (Ricord, note to John Hunter's Works, 1853).]

On the other hand, Dubois claimed a specific character for the affection {275} on the ground (1st) of the fact that in the majority of cases there was a syphilitic history in the parents; and (2d) that the eruption often coexists with well-known syphilitic lesions. This was supported by Cazenave, Danyan, Bouchut, Vidal, Ollivier, and others.[69] Diday, who devotes several pages of his interesting work on Infantile Syphilis to this subject, regards the eruption as simply a manifestation of a cachexia produced by syphilis,[70] founding this opinion on (1st) the absence of specific characters in the eruption; and (2d) that syphilitic pemphigus is a rare affection in the adult, if it occurs at all, so that to recognize it in the child would be to make a single exception to the general rule that "all the syphilitic eruptions of new-born children have their equivalents in those of adults." He explained the two cases which were then (1858) recorded of cures of pemphigus by mercury[71] by saying that it was the treatment of the diathesis, not of the disease, which caused the improvement. He acknowledges, however, the very frequent association of pemphigus in the child with syphilis in the parent, and says that it springs from the latter affection, "specially, but not specifically"--a rather wire-drawn distinction.[72]

[Footnote 69: Jullien (op. cit., p. 1005), after considering the opposing views as to the character of this eruption, says: "We have no hesitation in declaring ourselves in accord with Roger, Ollivier, Ranvier, Parrot, and others, and in distinctly separating from the specific affection the rare eruption known as simple pemphigus, sometimes epidemic, occasionally febrile, and appearing most frequently about three months after birth. We consider likewise that an evidence of congenital syphilis which is by no means doubtful is found in the bullous eruption seen at birth or within the first two weeks, comparatively frequent, and involving by preference the palms and soles. This opinion is based upon (1st) its appearance in children whose parents are known to be syphilitic; (2d) its association with syphilitic lesions of the lungs, liver, kidneys, thymus gland, etc.; (3d) its partial disappearance under mercurial treatment, and its reappearance when that treatment is discontinued."]

[Footnote 70: Op. cit., pp. 70-77.]

[Footnote 71: Depaul, Gaz. Méd. de Paris, 1851, p. 472, and Galligo, Gaz. Med. Toscana, 1852, p. 123.]

[Footnote 72: Trousseau (Clinical Lecture on Syphilis in Infants), after detailing a case in which there was some doubt as to the existence of hereditary syphilis in a child born alive, and in which case the previous pregnancy had resulted in a stillborn child at seven months, the body of the latter having been preserved in alcohol and exhibiting numerous traces of pemphigus, says: "So far as I was concerned, this demonstration did not amount to more than the establishing of a probability, and several physicians who participated in this indecision finally accepted a compromise. They considered that maternal syphilis had determined a sort of cachexia in the foetus which had led to an eruption of bullæ which was not specific. By accepting this too-facile hypothesis you will imprudently open a door which you will with difficulty be able to close."]

As these differences of opinion have been perpetuated to the present day, it has seemed to me proper to make this reference to their history, although I am strongly convinced that the progress of clinical and pathological knowledge enables us now to assert that although, as an exception, bullæ may be due to a profound cachexia not dependent on syphilis, yet that in the large majority of cases they are specific in their character.

The argument which always seemed to me the strongest, the fact that a similar eruption is almost--or quite--unknown in the adult, has been removed by the observations of Cornil, who has shown that it belongs properly with the papular rather than with the bullous eruptions, and should be classed with the roseola and papules of early syphilis--just where, from its clinical history, we should expect to find it. The raising of the epidermic layers is due chiefly to their delicacy, their slight resistance, and their previous immersion in the amniotic fluid--i.e. to {276} conditions which are peculiar to the skin shortly after birth.[73] He founds these very important opinions upon the autopsy of a child stillborn a little before full term, the mother being in the height of secondary syphilis. The child presented characteristic bullæ on the soles and palms. After hardening these were found to consist of the two layers of epidermis placed one above the other. Fig. 6 represents a bulla about one centimeter in diameter which was situated on the plantar surface of the great toe.

[Footnote 73: Cornil, op. cit., p. 203.]

If, then, we find an infant at birth or immediately after[74] presenting on the soles, the palms, the fingers and toes, or on the limbs, an eruption consisting of blebs more or less perfectly distended with a liquid which may be clear, cloudy, or bloody, circular or oval in shape, sometimes irregular, seated on inflamed, reddish skin, and surrounded by trifling areolæ, we may strongly suspect the presence of syphilis in an active and most menacing form. And this suspicion becomes a certainty if, in combination with such an eruption, the general cutaneous surface is yellowish or muddy in hue, is hard, dry, wrinkled, without elasticity or softness--owing to the absence of subcutaneous fat--and, for the same reason, is furrowed and wrinkled about the face, imparting an appearance of senility; if the child has a hoarse cry, a discharge from the {277} nostrils; and, of course, if there are at the same time other and unmistakable syphilodermata. This eruption is specially important, however, because upon the recognition of its specific character in cases of stillbirth, or in those in which the child survives only a few days--not long enough for the development of further symptoms--will depend the opinion as to the cause of death, which, whether expressed or not, will determine the future treatment of both parents during the interval and of the mother during the next pregnancy.

[Footnote 74: Non-syphilitic pemphigus is said to be never present at birth, nor until the child has become considerably exhausted by wasting from some defect of nutrition. It therefore does not appear until it is several weeks old. It then attacks the trunk in preference to the palms and soles.]

We may now consider the other symptoms of the secondary period in the child.

Coryza is one of the most characteristic, and at the same time one of the most important, of these in its influence on the health of the child. It is due to the same condition of the mucous membrane lining the nasal fossæ as manifests itself simultaneously or soon afterward on the skin in the shape of erythema, roseola, or papules; in other words, it is a hyperæmia with papillary infiltration. Now, on the skin this condition, except in so far as it indicates the presence of a grave constitutional disease, is of no special importance. In the nostrils of a sucking infant, already debilitated and impoverished by the anæmia of syphilis, and depending upon its nutrition for the continuance of the miserable flickering life which was its original endowment, the same condition assumes the gravest significance.

The excessive supply of blood to the parts induces a catarrhal condition which shows itself in a thin, watery discharge, which, as the child during sucking is compelled to breathe through the nose, is rapidly dried into crusts. These become adherent, fill up and lessen the channel for the passage of air, and in so doing add to the rapidity and force of the respiration through the nose, and thus increase the tendency to the deposit of these crusts. The peculiar nasal, noisy respiration of the child has given the affection the popular name of snuffles. As the child can no longer breathe, or can breathe only with great difficulty, while sucking, it takes the breast only to drop it again immediately on account of impending suffocation.[75] As the disease progresses ulceration occurs beneath the crusts, and often involves the entire thickness of the delicate mucous and periosteal layers underlying the thin bones of the nose; perforation of these bones results, sometimes with caries to such an extent as to cause an entire loss of the nasal septum, with flattening of the nose--a symptom comparable to one which sometimes occurs in the tertiary period of adults, but produced, as we have seen, by other causes. In adults syphilitic caries and necrosis are usually due to lesions seated primarily in the osseous or subperiosteal tissues; in the child, at least in this instance, these tissues are involved secondarily.

[Footnote 75: For an admirable description of the mechanism of this and other symptoms of coryza see Diday, op. cit., pp. 78-83.]

Erythema, or roseola as it is differently called, is apt to present itself about the second or third week[76] after birth. As in the adult, it begins upon the abdomen in the form of little oval, circular, or irregular spots, dull red in color and disappearing upon pressure. Later the color becomes deeper, the eruption extends to the trunk and limbs, and, as exudation and cell-proliferation succeed to simple capillary stains, it {278} ceases to disappear when pressed upon. It is often moist, owing to the thinness of the epidermis, sometimes excoriated. Occasionally it is confluent, and covers large areas with an almost unbroken sheet of deep-red color.

[Footnote 76: Bassereau gives an instance of its occurrence within three days.]

The diagnosis in the early stage is often difficult on account of the resemblance to the simple erythema of infancy. As the disease progresses, however, maculæ form here and there; the cell-infiltration involves the papillæ, several of which coalesce, forming flat papules; the nutrition of the superficial layers of the epiderm is interfered with, especially where it is thick, as on the palms and soles, and the eruption in those regions becomes scaly, and then the diagnosis is not difficult.

Papules and Mucous Patches.--In the ordinary evolution of the disease the next manifestation is usually the development of papules upon the general cutaneous surface and of mucous patches on the tongue, lips, and cheeks--probably also on other mucous membranes not exposed to examination. The papules are apt, for the reason already mentioned--the thinness and moisture of the skin--to be of the broad, flat kind, especially, as in the adult, in those regions where the elements of warmth and friction are superadded to the moisture, as in the folds of the skin about the genitalia, the neck, the flexures of the joints, etc. They are then moist, covered with a grayish secretion or a thin crust, and are in reality mucous patches. Occasionally they take on a little hypertrophy and develop condylomatous excrescences which closely resemble the simple acute condylomata of infants. In syphilis, however, the growth springs from a previously existing papule, which is not apt to be solitary, there being others in the neighborhood which will probably establish the diagnosis. The syphilitic condylomata also have a peculiar fetid discharge, resembling that of mucous patches and more or less characteristic.[77]

[Footnote 77: Van Harlingen, article "Syphilis" in the International Encyclopædia of Surgery, vol. ii. p. 560.]

Mucous patches in the infant are among the most important of the early syphilitic lesions--not to the child itself, because they do not materially affect its health, save in those exceptional instances where they are accompanied by a marked degree of stomatitis, and thus interfere with its nursing. Their importance is due to the fact that they are almost constantly present, and they are thus by far the most frequent vehicle of contagion from the child to its nurse or to others with whom it may come in contact. At times they do not differ materially from the same lesion occurring in the adult, but lose much sooner their epithelial investment (on account of the delicacy and comparatively slight attachment of the epithelium at this stage), and they then appear as oval or irregular red, slightly depressed spots, distinct or coalescing, ulcerating or oftener covered by a false membrane. They especially affect the angles of the mouth and the sides and dorsum of the tongue; and indeed their disposition to select the former situation constitutes a diagnostic difference between them and non-specific stomatitis which is to be found in the sulci between the gums and cheeks and on the gums themselves--locations rarely invaded by mucous patches.[78] When the latter are ulcerating or are concealed by diphtheritic membrane, and are situated on the tongue, they may be mistaken for either simple or parasitic stomatitis. The {279} diagnosis can often be made by the presence of other syphilitic symptoms--coryza, erythema, and especially papules. In their absence, however, it must be remembered that in simple stomatitis, the inflammation not being limited to special areas, the whole tongue is apt to be involved or a much larger portion of the buccal mucous membrane; and as there is no marked tendency to cell-proliferation in these cases, the accompanying exudation is apt to be serous or watery and to result in vesiculation--a condition never seen in syphilitic stomatitis. In the parasitic disease, too, the inflammation is less localized, there is more swelling and congestion, and the false membrane is said to be of a whiter color.

[Footnote 78: Bumstead and Taylor, op. cit., p. 750.]

No child that has even been suspected of having a taint of hereditary syphilis should be permitted to nurse at the breast of any one but the mother, to share its cup or nursing-bottle with other children, to receive the caresses of relatives or friends; and in this last restriction we would include the father, even if the suggestion[79] be true, that in the case of syphilitic children the protection from contagion probably extends to the male as well as the female parent. Paternity is sometimes a more doubtful problem than would seem probable, and even if the father were protected the husband might not be. The mucous patches, if any are found to exist, should be actively treated both locally and constitutionally, and during their demonstrable presence a most rigorous quarantine should be observed.

[Footnote 79: Hyde, op. cit. See p. 264.]

Syphilitic condylomata are due to hypertrophic changes in the papules, which under the influence of heat and moisture in certain regions coalesce and become more elevated. They vary in size from an eighth of an inch to a quarter or even a half of an inch in diameter. Their surface is flat and covered by a crust or by an offensive secretion. They are found most commonly about the anus or at the angles of the mouth.

Pustular Syphilides.--A little later in the secondary period, usually at about the sixth week, but sometimes much earlier, the papules become transformed into pustules, the change taking place slowly, so that if examined at any time after it has begun the child will present an eruption which is markedly polymorphic, showing here and there yellowish or reddish-yellow maculæ left after the absorption of the cell-element of certain papules, at other places beefy-red papules at the height of their development, or papules crowned by a ring of desiccated and desquamating epidermic scales, and in still other regions pustules in various stages of formation. Or the various formative stages of the pustules may be passed through so quickly that the eruption will be almost entirely pustular, few if any unmodified papules being discovered. The pustules may remain distended with pus for a considerable time, after which they may wither and slowly disappear or may rupture and leave ulcerated surfaces. A number of these ulcers sometimes run together and make extensive patches covered with thick, dark-colored crusts. These patches may resemble areas of impetigo or of impetiginous eczema, but in those affections the crusts are usually thinner and of a lighter color, and the skin beneath them is generally on a level with the surrounding surface, bright red and glazed; while under the crusts of the syphilide will be found a more or less depressed or excavated ulcer, often covered with pus. The diagnosis may indeed often be made by gently detaching and {280} raising one of the crusts and noting the character of the surface beneath. The erosion under the crusts of eczema heals over more readily and without leaving a cicatrix.

A so-called furuncular eruption[80] is said to appear at variable periods between the sixth month and the third year, but does not appear to me to be clearly differentiated from the large pustular syphilides with thickened and elevated bases on the one hand, or the ulcerating tubercular eruption on the other.[81] They are all so rare in hereditary syphilis, at any rate, as to have little clinical importance.

[Footnote 80: Bumstead and Taylor, op. cit., p. 750.]

[Footnote 81: The distinction between the two forms is usually manifest if the development of the lesions has been observed; but even this fails in regard to the tubercular eruption. They both occur at the same period; they both begin similarly, the furuncles as "small nodules in the corium," the tubercles as "deeply-seated papules or nodules;" they both run on to ulceration and pursue a chronic course (Van Harlingen, op. cit., p. 561).]

Iritis.--Another symptom of the secondary period, but of later development and of rarer occurrence than the syphilodermata which have been described, is iritis. In spite of its rarity this is extremely important, because it is frequently overlooked until it has reached such a stage that occlusion of the pupil results, and also because when it is recognized it constitutes an almost pathognomonic sign of syphilis.[82] This statement may now be made unhesitatingly, although for many years it was contended that iritis, and even the still more characteristic symptom keratitis, were only associated with syphilis as coincidences, the constitutional disease, when hereditary, having no causative relation to the local condition.

[Footnote 82: "When primary iritis occurs in syphilis in young children it is almost always due to syphilis" (Soelberg Wells, Treatise on Diseases of the Eye, Philada., 1873, p. 173).]

To Mr. Hutchinson belongs the credit of having first clearly developed the specific character of this trouble,[83] which, on account of the mildness of the attendant symptoms, is often overlooked. The sclerotic zone of congestion so marked in the adult, and therefore so valuable a diagnostic sign to the general practitioner, is very slight, sometimes absent; and as a consequence the attention of neither parent nor physician is attracted to the condition until in the more serious cases it has done irreparable mischief. In milder cases, particularly where the child is under mercurial treatment for concomitant symptoms of syphilis, it may run its course and escape notice altogether;[84] and it is possible that owing to this fact the rarity of the affection has been overestimated. It is also possible that in such cases changes occurring at this time may in some instances lay the foundation for some of the deeper-seated ocular troubles of later life.

[Footnote 83: Med. Times and Gazette, 1860, July 14; Ophthalmic Hospital Reports, vol. i. pp. 191, 226; A Clinical Memoir on Certain Diseases of the Eye and Ear consequent on Inherited Syphilis, London, 1863. In the introduction to this volume Mr. Hutchinson states that acute iritis dependent on hereditary syphilis was first described in connection with its true cause by Mr. Lawrence, but, as from the date of that gentleman's first case (1830) up to 1863 but six cases had been recorded, the announcement had made but little impression on the profession.]

[Footnote 84: "In the cases of this form of iritis which are seen in ordinary eye-practice much damage has often been done by occlusion of the pupil and deeper mischief. Probably many of the slighter cases escape the notice of the parents and are not brought to the surgeon" (Mr. Edward Nettleship. See Hill and Cooper, p. 271).]

If, however, attention has been attracted to the eyes, the diagnosis is not usually difficult. The pupil is irregular, especially under atropia; {281} there are streaks of lymph, dulness, swelling, change of color, and on very careful inspection a faint pink zone may be seen in the sclerotic. The conjunctiva and cornea are generally clear.

Mr. Hutchinson's analysis of the twenty-three cases reported by him is still of interest as furnishing reliable data for prognosis. The average age at the time the iritis commenced was five months and a half. The oldest was sixteen months at the time of the outbreak, the youngest six weeks. In twelve cases but one eye suffered; in eleven both were affected. In seven cases (ten eyes) the cure was complete; in two or three other cases very slender adhesions remained; in twelve cases, in nearly all of which the patients came under care only at a late period of the disease, one pupil was permanently occluded by organized false membrane. In nearly all, coincident symptoms of syphilis of the skin or mucous membranes were present. Of the thirteen cases in which alone a history of the family is recorded, the affected infant was the only living child of his parents in twelve instances. In the only case in the whole series in which it is stated that there were other living children the mother had lost four infants out of seven live births.

The prognosis depends on the stage at which they come under treatment. The lymph if recent, no matter in what quantity, will probably be absorbed under mercurial treatment, which will often be of great benefit even in those cases in which a certain amount of organization has occurred.[85]

[Footnote 85: It will not be uninteresting, perhaps, to append the aphorisms regarding iritis in infants which Mr. Hutchinson at that time enunciated: 1. The subjects of infantile iritis are much more frequently of the female than the male sex. 2. The age of five months is the period of life at or about which syphilitic infants are most liable to suffer from iritis. 3. Syphilitic iritis in infants is often symmetrical, but quite as frequently not so. (In his article in Reynolds's System of Medicine, written in 1866, three years later, but revised in 1870, he describes it as "usually symmetrical," vol. i. p. 444.) 4. Iritis, as it occurs in infants, is seldom complicated, and is attended by but few of the more severe symptoms which characterize the disease in the adult. 5. Notwithstanding the absence of phenomena of acute inflammation, the effusion of lymph and the danger of occlusion of the pupil are usually very great. 6. Mercurial treatment is most signally efficacious in curing the disease, and, if recent, in procuring the complete absorption of the effused lymph. 7. Mercurial treatment previously adopted does not prevent the occurrence of this form of iritis. 8. The subjects of infantile iritis, though often puny and cachectic, are also often apparently in good condition. 9. Infants suffering from iritis should always show one or other of the well-recognized symptoms of hereditary taint. 10. Most of those who suffer from syphilitic iritis are infants born within a short period of the date of the primary disease in their parents.]

We have now a group of symptoms characteristic of the secondary period of syphilis, or that extending from birth, or much more commonly from the age of three or four weeks to about the end of the first year. The syphilitic child during this time has several or all of the following symptoms: Coryza with snuffles; an erythematous, papular, or pustular eruption on the skin; mucous patches on the lips, tongue, cheeks, etc.; a marked tendency to general wasting; a hoarse cry or cough; senility of aspect; iritis. The majority of syphilitic children born alive die during this stage.

Before its termination, sometimes even at birth, other lesions have been noticed (especially those affecting the liver), which, however, may better be described in connection with the special organ or organs involved.

Succeeding this stage--i.e. beginning in about a year or eighteen {282} months--comes an intermediate period, which extends to second dentition, to puberty, or even much later, and which is characterized rather negatively--that is, by the absence of symptoms--than otherwise. The evidence of the general diathesis will of course be present in the shape possibly of malnutrition, stunted growth, or retarded development, perhaps shown in the weazened or withered face, the sunken nose, the pallor of the skin, the premature loss of the upper incisor teeth or the malformation of the others if they have erupted.

There is but little tendency to recurrence or relapse of any of the secondary symptoms; and in certain cases, not a very small proportion, in which these symptoms have been light and have been well and thoroughly treated, this stage extends throughout life; or, in other words, as is frequently the case with the adult who has followed a proper course of treatment, the disease appears to terminate with the secondary stage. In other cases, however, it recurs, and the symptoms which it then presents may be taken up in connection with the different organs or tissues involved.

Syphilis of the ear is for obvious reasons not often discoverable until the patient has reached an age at which interference with the function of hearing becomes a noticeable phenomena. The only symptom likely to attract attention during the stage of inherited syphilis which we are now considering is a catarrh of the middle ear, which may have for its starting-point some inflammation, ulceration, or mucous patch of the pharynx, causing a temporary or permanent occlusion of the orifices of the Eustachian tubes.[86] This may lead to perforation of the membrana tympani, purulent infiltration of the mastoid cells, etc., and when accompanied by an otorrhoea which attracts attention to the ear will be easily discovered by the physician. These cases are, however, exceptional, otorrhoea only being present in nine out of Hutchinson and Jackson's[87] one hundred cases of inherited syphilis, and consequently but little is known about the frequency or gravity of lesions of the auditory apparatus in the secondary stage of this form of syphilis.[88] The changes which occur later on are chiefly those which involve either the nerves themselves or their distribution in the labyrinth.

[Footnote 86: Bäumler, Ziemssen's Cyclopædia, vol. iii. p. 226.]

[Footnote 87: Hutchinson and Hughlings Jackson, Med. Times and Gaz., Nov. 23, 1861.]

[Footnote 88: Schwartze (quoted by Hill and Cooper) found also that otorrhoea was a rare complication in deafness from syphilis.]

The affections of the middle ear and Eustachian tube are said to be contemporaneous with the keratitis which appears in the neighborhood of puberty,[89] while those of the nerve are somewhat later in point of time, and are almost always conjoined with retinitis, choroiditis, and optic neuritis. As usual when investigating or describing any subject relating to syphilis, Mr. Hutchinson's opinion and observation must be detailed. In 1863 he wrote[90] that it was only recently that he had thought of specially investigating the disorders of hearing in reference to hereditary taint, having had his attention called to a peculiar form of deafness, usually symmetrical, passing rapidly through its different stages and {283} unaccompanied by any marked degree of pain or any external disease. He then reported eighteen cases of which he had notes. The oldest of these patients was twenty-seven, the youngest eight--the average time of development of the deafness from twelve to fifteen. Although the membrana tympani was in no instance quite normal, in none were there found adequate changes to account for the deafness. In all the Eustachian tubes were pervious. In nearly all the disease was symmetrical. This fact, together with the absence of discoverable lesions of the external or middle ear, seems to point conclusively to disease of the nerves themselves, or at least to a central cause.[91] He adds: "With regard to the prognosis of heredito-syphilitic deafness, I believe that it is very unfavorable. When the disease was progressive I have rarely witnessed any permanent improvement or arrest. In most it has gone on to total loss of hearing, and this in several instances in spite of the cautious use of specific remedies almost from the beginning. From six months to a year would appear to be the usual time required for the completion of the process and the entire abolition of the function."[92]

[Footnote 89: Purves, Guy's Hospital Reports, 1875, p. 564; Pritchard, British Medical Journal, April 21, 1877.]

[Footnote 90: Clinical Memoirs on Certain Diseases of the Eye and Ear consequent on Inherited Syphilis, London, 1863, pp. 182, 183.]

[Footnote 91: In the Lancet for Jan. 16, 1875, he reports a case of total deafness in a young woman of seventeen which had come on in ten months without pain or otorrhoea. He believes the disease of the organ of hearing to be parallel with those cases of choroiditis disseminata or of optic neuritis in which blindness is produced without pain or any external evidence of inflammation, and which are distinctly and positively associated with inherited syphilis.]

[Footnote 92: Mr. Hinton, in his edition of Toynbee's work on Diseases of the Ear, states that at Guy's Hospital, of his aural patients, one in twenty is affected with deafness due to heredito-syphilis; that it usually makes its appearance between the tenth and sixteenth years; and that the great majority of the cases which he has seen have been females. He adds: "Patients suffering from this disease may, as a rule, at least when young, be at once distinguished by the amount of deafness which they exhibit. I know no other affection except fever which in a person under twenty brings on a deafness so rapidly and so nearly complete. In the course of a few weeks a girl previously hearing well will, without pain or known cause, become unable to distinguish words." In one of Dalby's cases total deafness came on in three weeks, previous to which hearing was normal. According to Pierce, the deafness is most apt to manifest itself between eleven and eighteen years of age. Troeltsch says that "l'audition du diapason par le vertex" is lost at an early date after the beginning of the disease, and that there are also often concomitant affections of the nose and pharynx.]

Dalby[93] is said to regard syphilis as, next to scarlatina, the most fruitful cause of deaf-mutism as it occurs in children born with good hearing powers. "The patient usually becomes deaf in early childhood--after he begins to talk--or between this period and puberty."[94]

[Footnote 93: The Lancet, Jan. 22, 1876.]

[Footnote 94: Bumstead, op. cit., p. 734.]

Syphilis of the Liver.--In 1852, Gubler published an account of the general appearances in syphilitic disease of the liver in new-born children, which was distinguished especially by increase in size and weight. This increase depended, as might be expected in this stage--that of general cell-proliferation--upon a proliferation of cells from the connective tissue between the acini, or from the adventitia of the interlobular vessels, this growth becoming transformed into connective tissue.[95] The change is quite analogous to what is taking place at the same time in the skin, the mucous membranes, and other tissues. Wilks has also described[96] a form of syphilitic disease of the liver which corresponds to that of Gubler, and in which the whole organ is infiltrated by a new fibrous tissue, producing a uniform and general hardening.

[Footnote 95: Bäumler, op. cit., p. 186.]

[Footnote 96: Trans. Path. Soc., vol. xvii., 1866.]

{284} [Illustration: FIG. 8. Section of an old gumma of the liver. a, a. Central caseous tissue of the gumma. v', v'. Its vessels. l. Boundary between the central portion and fibrous zone; this line of demarcation is marked in places by an opening or cleft. t, t. Connective tissue of the fibrous zone which entirely surrounds the central part. v, v. Small vessels of this zone. c. An arteriole of the fibrous zone. f, f. Quite large biliary vessels included in the fibrous zone. t'. Fasciculi of connective-tissue fibres running parallel with the surface of the caseous part. At b and d the fasciculi of fibres of the fibrous zone penetrate into the central caseous part. e, e. Tissue of hepatic cells interrupted by bands of fibrous tissue, m, m. X 12.]

As described by Gubler,[97] the liver in such children is hypertrophied; hard, resistant to pressure, so that it cannot be indented; elastic, so that it rebounds; creaks, but does not bleed, when it is cut into, and presents the yellow color and the semi-transparence of flint. There are seen on a yellowish ground a number of small white granulations like grains of wheat, which a histological examination shows to be formed by an accumulation of embryonic cells in the spaces which separate the hepatic acini. Injections reveal the fact that the vascular network has become almost impenetrable, the capillaries obliterated, the larger vessels diminished in calibre. Fibro-plastic matter is found throughout the organ in large quantity. In consequence of these conditions--the compression of the hepatic cells and the destruction of the vessels--the secretion of bile is stopped, and the gall-bladder is found after death to contain a pale-yellow liquid consisting of bile mixed with an excess of mucus. This form of hepatitis has thus far been observed almost exclusively in infants. Cornil {285} says[98] that he has had frequent occasion to examine such cases of hepatic syphilis, and describes them as follows: "The hepatic acini, in the normal state, are in contact except at the prismatic spaces which are formed by their union--spaces in which the capsule of Glisson forms an envelope to the afferent portal vessels of the lobuli. It is in these spaces that the round lymph-cells form and collect into small nodules representing microscopic gummata. The cells at the centre of the new formation are sometimes granular. This neoplasm is seated about the ramifications of the portal veins, which in consequence also present thickened walls with newly-formed cells in their external tissues. The small granules above mentioned are not always visible to the naked eye, and in their places are only seen, about the perilobular capillaries of the portal vein, an excessive number of embryonic cells." In addition to this interstitial sclerosis or interstitial infiltrating hepatitis there is an inflammation of the liver depending upon the presence of gummata--gummous hepatitis--which occurs in two forms: one in which very small and very numerous nodules are present, situated along the course of the fibrous seams, the prolongation of the capsule, and another in which there are two or three large circumscribed tumors. This form of hepatitis is always accompanied by the interstitial form, although the latter may be only slightly developed.[99] The gummata, though not infrequently found in the liver of new-born children, are more likely to develop later, at from about the eighth to the twelfth year.

[Footnote 97: Mémoires sur une nouvelle Affection de Foie, and Gaz. Méd. de Paris, 1852.]

[Footnote 98: Op. cit., Am. ed., p. 370.]

[Footnote 99: It does not differ essentially, either pathologically or clinically, from the same lesion in adults.]

Rochebonne[100] describes the following symptoms of syphilitic hepatitis in infants: A deep wine-colored venous stain and oedema of the lower extremities, often accompanied by pemphigus; ascites due to mechanical obstruction of the circulation, as in cirrhosis; a more or less pronounced chloro-anæmic appearance of the face; and the presence in the urine of albumen and hæmato-globulin. Vomiting may occur, and constipation alternating with diarrhoea has been observed. Icterus, symptomatic of the affection, has not been observed.

[Footnote 100: Quoted by Bumstead and Taylor, p. 758.]

Bäumler says:[101] Implication of the peritoneal coating of the liver may be recognized by the pain in the hepatic region. In new-born children--unless, possibly, there may be some enlargement of the liver--the only local symptoms, often, are those due to peritonitis--screaming, drawing up of the legs, vomiting. In those cases it is not rare for the peritonitis to become diffuse.[102]

[Footnote 101: Op. cit., p. 194.]

[Footnote 102: In an article on "Inherited Syphilis" in the British and For. Medico-Chirurgical Review, 1875, p. 28, it is said: "Of the liver the lesion consists in enlargement and induration of the organ in whole or in part, due to the development of fibro-plastic material between the cells of the acini, with obliteration of the vessels and interference with the secretion of bile. This condition is generally doubtful during uterine life, and is rapidly fatal. The symptoms are vomiting, diarrhoea, and tympanitis, but, strange to say, no jaundice. The enlarged and indurated organ may be felt by palpation. It is probably in this connection that the peritonitis described by Simpson as occurring in inherited syphilis is found."]

Hill says:[103] "The symptoms are mainly those of functional derangement of the organ, with alteration of its bulk."

[Footnote 103: Op. cit., p. 163.]

Hutchinson[104] has described cases in which in young persons the subjects {286} of hereditary syphilis there has been great hepatic enlargement which has subsequently wholly disappeared. He finds it difficult to believe that there is any kind of gummous growth in such cases, and feels obliged rather to fall back upon the hypothesis of mere vascular turgescence. In one such case the liver occasionally was so large as to be visible as the patient lay on his back in bed.[105]

[Footnote 104: Path. Transactions, 1877, p. 309.]

[Footnote 105: Illustrative cases of this condition may be found in the Med. Times and Gazette, Dec. 22, 1877.]

It seems much more likely that the enlargement is due to an exceptionally active cell-proliferation, which does not, however, go on to organization, but may be just as susceptible of absorption and resolution as are the papules or maculæ of the skin. A portion of the enlargement may be due to a passive congestion caused by the presence of this cell-accumulation.[106]

[Footnote 106: Barlow (Path. Trans., 1877, p. 355) has suggested that the engorgement is only a preliminary stage of the fibrous thickening, and may disappear either with or without leaving permanent contractions or adhesions in its wake.]

As to the diagnosis of hepatic syphilis in infants, I am disposed to agree with Cornil, who says:[107] "The symptoms are null, or they are identical with those of local and general troubles so often observed in children who have poor or insufficient nourishment. The only physical sign which properly belongs to hepatic syphilis is, when it exists at all, increase in the size of the liver."

[Footnote 107: Op. cit.]

Syphilis of the Bones.--Until the publication in 1870 of the researches of S. Wegner,[108] an assistant of Prof. Virchow, diseases of the osseous system due to hereditary syphilis were either ignored or denied by the various writers upon this subject.[109] Valleix, Bargione, Ranvier, and Guéniot had indeed recorded cases of bone disease occurring at the points of junction between the epiphyses and diaphyses and in the costal cartilages, but it remained for Wegner first fully to describe the pathological changes which occurred there, and to differentiate them from those due to rickets or scrofula. His memoirs recognized three stages of alteration in the long bones:[110] 1st. While in the normal state the boundary of the hyaline cartilage is distinctly marked by a line which indicates the direct transformation of the cartilaginous tissue into a spongy tissue, the unaided eye being unable to distinguish a spongio-calcareous layer, in new-born syphilitic children, on the contrary, the bones are seen to have a spongio-calcareous layer interposed between the bone and cartilage, measuring two millimeters in thickness. This is a zone of calcifying cartilaginous material more extensive than in the normal state. 2d. These same changes become more distinct and more extensive. The unnaturally thick layer of calcareous material continues to grow. There is proliferation of the cartilaginous trabeculæ, abundant calcification of the cartilage, too early and irregular ossification of the intercellular substance {287} of the cartilage, and at the same time an arrest of the normal formation of bone which should be going on from the epiphysial cartilage. 3d. There is now added, by extension of these processes, a thickening of the perichondrium and periosteum at the extremities of the long bones and at the junction of the ribs with the costal cartilages. In consequence of the interference with nutrition occasioned by these changes atrophy and fatty degeneration of the cartilage-cells occur, and they form between the epiphysis and diaphysis a necrosed mass which irritates the living bone. This causes osteo-myelitis, which frequently results in a separation of the epiphyses. Occasionally pus is produced in such quantity as to perforate the periosteum, escape into the surrounding tissues, and become superficial. He terms the entire process an osteo-chondritis.

[Footnote 108: Virchow's Archiv, 1870, B. 50, S. 305: "Ueber hereditäre knochen Syphilis bei jungen Kindern."]

[Footnote 109: Diday says: "Affections of the bones are so rare in children with inherited syphilis that the annals of medicine scarcely offer five or six well-authenticated cases of caries or periostitis" (op. cit., p. 83). Referring to this statement, Mr. Hutchinson remarks: "So different has been my own experience from this that I may say that we are scarcely ever without a severe example of it in the wards of the London Hospital" (Illustrations of Clinical Surgery, London, 1875, p. 47).]

[Footnote 110: Cornil, op. cit., p. 282 et seq.]

Waldeyer and Köhner,[111] after examining twelve cases, confirm in the main these investigations of Wegner, but interpret the changes as arising rather from the formation of a gummous tissue between the epiphysis and diaphysis than from an osteo-chondritis. The tissue-death which occurs later, the atrophy of the cells, etc., they compare with the same modifications observed in syphilomata.

[Footnote 111: "Beiträge zur Kenntwiss der hereditäre knochen Syphilis," Virchow's Archiv, B. 55, S. 367.]

Parrot[112] in a number of exceedingly valuable papers has repeated and greatly extended these observations. He places especial importance upon the formation of osteophytes, which, he says, in the first stage envelop the diaphyses of the long bones, especially at their inferior extremities. In the succeeding stage the new bony layers are more porous; a gelatinous degeneration affects the epiphysial cartilage and the spongy bones at a point where they are in contact; the epiphyses tend to separate from the diaphyses. This solution of continuity results in a characteristic pseudo-paralysis, with curvatures, abnormal twistings, and preternatural mobility of the bones, with loss of the power of locomotion. Then the osteophytes increase in size by the formation of several layers, thus enlarging the inferior extremities of the long bones. He describes the general process as consisting, first, of a periosteo-genesis--a formation of osseous tissue from the periosteum; next of a chondro-calcosis--a calcareous incrustation of cartilage; and finally of a gelatiniform degeneration and softening of the bone, with diaphyso-epiphysial disjunction.[113]

[Footnote 112: Société de Biologie, June 1, 1872; Société anatomique, 1873, p. 92; Archives de Physiologie, 1876, vol. iii. pp. 138, 139; Revue mensuelle de Médecine et de Chirurgie, 1877; Pathological Trans., 1871, vol. xxx. p. 339, etc., etc.]

[Footnote 113: Cornil (op. cit.) coincides in the main with this description.]

Taylor[114] sums up the results of his observations as follows: "In the first stage we have a simple hyperplasia of cells with irregular deposition of lime salts; in the second, an intensification of this condition; and in the third, a new element--namely, the abnormal proliferation of all the elements of the tissues, with an infiltration of granulation-tissue into the medullary spaces following the vessels."[115]

[Footnote 114: Syphilitic Lesions of the Osseous System in Infants and Young Children, New York, 1875, p. 134.]

[Footnote 115: Verraguth (Archiv für Path. Anat.) describes the first step as an excessive formation of vessels in the cartilage and a corresponding overgrowth of the cellular elements. This becomes inflammatory, and constitutes a primary syphilitic chondritis, the changes in the medulla of the bone being degenerative and secondary to the affection of the cartilage. Still other observers have described the process, each with minor modifications; but as they are of no clinical importance, it does not seem worth while to quote them.]

{288} We see, then, that, setting aside minor points of difference, these observers all coincide in describing this condition as one essentially of the nature of syphilitic bone troubles with which we are familiar in the acquired form of the disease, consisting primarily and throughout of an unnatural accumulation of cell-elements, which in the later stages by their pressure produce various degenerations of surrounding structures, and which, as they occur during the process of bone-formation, are accompanied by irregular and abnormal deposition of lime salts. They especially affect the regions mentioned--the junctions of the epiphyses and diaphyses--because at that time those points are the seat of great physiological activity. Syphilis, indeed, throughout its entire course is notably subject to similar influences, as one example of which I may instance the preference displayed by the periostitis which results in nodes or in caries for the subcutaneous bones, the tibia, clavicle, cranium, etc.; or, in other words, for those which are subject to frequent traumatisms--trifling, perhaps, but sufficient to determine a slight hyperæmia, which is followed by abnormal cell-proliferation or accumulation.

The symptoms which obtain in this condition of syphilitic osteo-chondritis are as follows: The child may be attacked during intra-uterine life, and in that event the osseous lesions will probably be coincident with other syphilomata and with placental disease of sufficient gravity to destroy life.[116] If the child is born alive, the first development of the disease will probably be noticed as a swelling at the diaphyso-epiphysial junction of one of the long bones, which in the emaciated subjects of hereditary syphilis is often visible, and can always be discovered by palpation. The bones most frequently attacked are the humerus, radius and ulna, tibia and femur, but the clavicle, ribs, sternum, and bones of the metatarsus and metacarpus are also often involved, and much more rarely the frontal and parietal. The more pronounced the syphilis of the parents, or the nearer the date of conception to the time at which their infection occurred, the more probable is it that several bones will be affected, and the more unfavorable the prognosis as respects the life of the child. Indeed, it has been noticed that "in stillborn infants and in those dying soon after birth the majority, or even all, of the long bones are affected."[117]

[Footnote 116: Pollnow found osteo-chondritis in 35 out of 50 syphilitic foetuses (Der Hydrops Sanguinolentes foetus, Berlin, 1874, quoted by Hill and Cooper, op. cit., p. 352).]

[Footnote 117: Bumstead and Taylor, op. cit., p. 767.]

The swelling is found to consist of a ring or collar which more or less completely surrounds the bone, is apt to be smooth rather than irregular, and when two bones situated near to each other are simultaneously affected may conjoin them. This condition persists during the first stage of pathologists, and passes with greater or less rapidity into the second stage, in which the swelling, the cell-proliferation, reaches its height. This may take, in cases uninfluenced by treatment, several weeks or even months. Under the use of mercurials and iodide of potassium they usually subside rapidly. During this second stage, however, owing to the proximity of the swellings to the joints, a moderate amount of synovitis is often present. This affects chiefly the elbow and the knee, but may appear in any joint. It is also readily influenced by specific treatment and well-regulated pressure.

{289} When the third stage is reached, or that of the formation of granulation-tissue, with degenerative changes of the cartilages and of the bones themselves, deformity often becomes more marked. There are unnatural curves or angles in the bones, with more or less complete separation at the point of junction. Where many bones are affected in this way, the resulting deformity is extreme and the patient may be absolutely powerless, a condition of pseudo-paralysis supervening in which the limbs lie motionless or swing about like the arms or legs of a doll when the child is carried.

When the swelling does not undergo absorption, the superjacent tissues sometimes become involved, abscesses form and make their appearance externally, extensive necrosis of the shaft of the affected bone takes place, and the little patient usually dies of hectic, pyæmia, or exhaustion. When the cranial bones are involved, the disease is apt to limit itself chiefly to the stage of osteophytic formation, the immovability of the bones probably favoring the organization of the new cell-growth rather than the production in it of inflammatory changes. The growths are met with chiefly in older children than those affected with the form of osteo-chondritis just described; they affect the periphery of the liver, and are found most usually around the anterior fontanel, and later on the parietal and frontal eminences. The sutures are sometimes completely soldered together.[118] The osteophytes vary in thickness from a quarter of an inch to an inch, or are even larger.[119]

[Footnote 118: In a case reported by Barlow it was not possible at the autopsy to discover the point of union (Path. Transactions, 1879, p. 339).]

[Footnote 119: These conditions may all result in a child the subject of acquired syphilis, but are apt to be milder, to involve fewer bones, and to yield more readily to treatment. This would of course be expected, inasmuch as the same difference in favor of the acquired form, as compared with that which is inherited, extends to all the lesions. As Diday succinctly expresses it: "In the one case the poison vitiates only the elements of nutrition; in the other it vitiates at the same time those of formation and those of nutrition." It would exceed the limits of the present article to describe acquired syphilis in children.]

The most important differential diagnosis to be made in these cases is between the rachitis of young children and the form of syphilis in question. Much difference of opinion still exists as to the relation between these diseases, syphilis being claimed, on the one hand, as having in the majority of cases a definite causative influence, while, on the other, the existence of this relation is denied. When we come to contrast the pathology of the two diseases, we can readily understand why they should be confounded, the minuter changes which occur being essentially the same--viz. cell-proliferation and accumulation, with subsequent inflammatory changes, associated with irregular deposits of lime salts.

Compare, for example, the description of the pathology of bone diseases in inherited syphilis already given (pp. 287, 288) with the following terse summary of the changes which take place in rickets in cases where no suspicion of syphilis exists, either ancestral or acquired: "The changes are more distinctly noticed at the epiphyses than in the diaphyses. Instead of the regular stages and distinct boundaries observed in the normal development of bone, there is a singular disorderly commingling of the exaggerated cartilage-proliferation and transition substance, with calcification. The cartilage-cells, stimulated to excessive multiplication, are transformed, some into bone-corpuscles, some into medullary cells, {290} and others into connective-tissue forms. The same process is in active operation in the deep periosteal layers, the material accumulating to such a degree as to add much to the thickness of the shaft."[120]

[Footnote 120: Agnew's Surgery, vol. i. p. 1030.]

The points of resemblance are manifest, just as they are between a syphilitic and a variolous pustule, but they end in both cases when we come to study the evolution of the phenomena either from an anatomical or from a clinical standpoint. They may be expressed as follows in tabular form:

OSSEOUS LESIONS DUE TO INHERITED | RICKETS. SYPHILIS. | | The swellings, particularly those| Rarely appear before six months, of the long bones, show | generally still later. themselves at or soon after | birth. | | A history of syphilis or evidence| No such history necessarily. of existing syphilis in one or | both parents. | | Preceded or accompanied by | No such prodromata. snuffles, coryza, and cutaneous | and mucous lesions. | | No such prodromata in most cases.| Pallor, restlessness, sweating, | nausea, diarrhoea, etc. | constitute a combination of | symptoms which often precede the | bone disease. | Cachexia absent or moderate. | Cachexia marked. | Physiognomical peculiarities of | Not present as a group. syphilis present.[121] | | Circumscribed tumors on frontal | Cranial bones thickened in spots, and parietal bones, rarely on | usually upon the occiput. occiput. | | Ribs not markedly affected. | All or nearly all involved. | Swellings on long bones or | Extremities symmetrically extremities irregular. | enlarged. | Disease of ribs, when existent, | Nearly always so. not ordinarily coincident with | that of other bones. | | Fontanels close at usual period. | Closure delayed. | Other syphilitic symptoms | Syphilitic symptoms absent. present; enlargement of | phalanges, metatarsal bones, etc.| | Often accompanied by sinuses, | Little external or surrounding synovitis, abscesses, cutaneous | involvement. ulcers, etc. | | Generally disappears by | Usually leaves some bending of resolution, without leaving any | shaft and distortion of the permanent change. | neighboring joint. | Mortality among children in whom | Much less. many bones are involved is very | great. | | Specific treatment useful. | Of no benefit. | In the first stage there is an | This is less marked. There is exuberant calcification of the | formed, instead, a soft and ossifying cartilage, causing | non-calcified osteoid tissue. necrosis of the new-formed tissue| and a consecutive inflammation, | which terminates in the | separation of the epiphyses.[122]|

[Footnote 121: See p. 313.]

[Footnote 122: This table is founded on one which I added to the translation of Cornil made by Dr. Simes and myself, and is compiled chiefly from the excellent work of Dr. Taylor already alluded to.]

The diagnosis of the bone lesions of hereditary exostosis can readily be recognized in a short time by noting the fact that they are stationary, {291} even if their later appearance, larger size, the absence of syphilitic history or symptoms, and the resistance to specific treatment left us in doubt.

The diagnosis from accidental separation of the epiphysis, or from fractures, may be made from the history of the case.

In cases of separation of the epiphysis, complicated with suppuration, sinuses, etc., the trouble may be mistaken for a similar condition due to non-specific inflammation. In all the recorded instances, however, the latter has occurred much later in life, is attended with much more acute inflammatory symptoms, lymphangitis, etc., and is of course without concomitant symptoms of syphilis. In both these cases there is a decided osteo-periostitis, and as so much depends on the early and vigorous use of specific treatment, it may be worth while to contrast the two forms of the disease.

SYPHILITIC OSTEO-PERIOSTITIS. | NON-SPECIFIC OSTEO-PERIOSTITIS. | Occurs in infants under three | No instance of its occurrence in months of age. | children under one year of age. | History of syphilis in child and | No history of syphilis; sometimes its parents. | a history of traumatism. | Implication of other bones. | Usually confined to one bone. | Coincident with the development | Coexists with the ossification of of the shaft of the bone. | the epiphyses. | Other lesions of syphilis: nodes,| No such symptoms. skin eruptions, etc. | | All the local symptoms | Pain, redness, and swelling very comparatively mild. | marked. | Disease sharply localized. | Involves neighboring parts. | Lymphatics of limb unaffected. | Lymphangitis present. | Beneficial effect of specific | No such effect. treatment if employed early.[123]|

[Footnote 123: Cornil, op. cit., p. 274.]

Syphilitic dactylitis in the inherited variety of the disease, as in the acquired, consists of two varieties. The one of these which usually appears earlier involves chiefly the periosteum and the fibrous and integumentary structures surrounding a joint, usually a metacarpo- or metatarso-phalangeal articulation, involving a phalanx, and is characterized by slow, almost painless, swelling and discoloration of the affected member. (Fig. 9.) This is due to a gummous infiltration which, after absorption under proper treatment, leaves the toe or finger temporarily stiff, but not permanently disabled.

The second form is a specific osteo-myelitis, with periostitis, coming on later, and often destroying the bone or the articulation involved. (Fig. 10.)

The absence of acute inflammatory symptoms in the first variety distinguishes it from paronychia, whitlow, and gout. Rheumatoid arthritis begins in the joints, is associated with other symptoms; deformity of the fingers comes early in the disease, and there is a teno-synovitis with contraction.

The second variety might be taken for enchondroma or exostosis, but these growths increase much more slowly, involve only a limited portion of the bone, are of greater density, and are much more strictly circumscribed.

As a rule, especially in cases which are recognized early and treated {292} actively, the prognosis is good. Iodide of potassium should be used in combination with mercury.

Syphilis of the Teeth.--Syphilis of the teeth has its chief interest to the general practitioner from its very important bearing on diagnosis. As manifesting itself at an age when the child is not apt to present the active and unmistakable cutaneous and mucous lesions of the disease, and when, consequently, its recognition is often extremely difficult, this diagnostic importance is greatly increased.

The teeth of the first dentition, although exhibiting the usual signs of interference with nutrition in their irregular development, opaque and chalky enamel deficient in quantity and unevenly distributed, soft and friable dentine, incongruity of size individually and relatively, and proneness to decay, do not often display any distinctive evidence of syphilis. {293} The same conditions may, and often do, depend on other causes, and are commonly associated with various cachexiæ--the strumous, gouty, rheumatic, rachitic, etc.--and even with other slighter ailments tending to produce imperfect assimilation and malnutrition.

In the permanent teeth, likewise, the same condition may be due to the same causes. Stomatitis, however produced--by mercury, by gastro-intestinal derangements, by local irritation of any kind--is apt to result in imperfectly organized dental structures. Mercurial teeth, for example, are usually irregularly aligned, horizontally seamed, honeycombed, craggy, malformed, of an unhealthy dirty yellow color, separated too widely, and deficient in enamel.[124] The diseases of childhood, especially the eruptive fevers, eclampsia, typhoid fever, etc., by temporarily arresting or greatly interfering with nutrition during the developmental period of the teeth, often cause horizontal furrows across their crowns, which are, of course, persistent throughout life, and mark indelibly the influence of such disorders on all the formative processes.

[Footnote 124: The latter defect is particularly noticeable on the cusps of the sixth-year molars. (See note on "Syphilis of the Teeth," by Dr. James W. White, in Am. ed. of Cornil, pp. 287-290.) The discussion as to the effect of mercury in producing the condition of the teeth known as honeycombed is still going on, but the evidence seems to point clearly to a direct connection with the administration of mercury in infancy, either for syphilis or in excessive doses as a purge, or in some of the teething powders, which often consist of calomel and opium. Mr. Hutchinson, at a meeting of the Odontological Society (see Proceedings for 1877, p. 249), gave an interesting description of the way in which the supposition was arrived at. Lamellar cataract is a disease which affects the eyes of children who have suffered from convulsions: it was noticed that in cases of that form of catarrh there were also honeycombed teeth, and it was thought that the convulsions, the cataract, and the honeycombed teeth were all due to the same unknown cause. At last a few exceptions were found--patients with cataract, but with good teeth, and then some who had had convulsions only, and yet had honeycombed teeth; lastly, it was noticed that most of the patients had been treated with mercury. So it came to be recognized that the honeycombed teeth were only accidentally associated with the cataract, and that they were, in fact, the result of the mercury which had been given to cure the convulsions. The same gentleman figures a case of this disease in his Illustrations of Clinical Surgery (London, 1875), and thus describes it (p. 55): "The present state of his permanent teeth is so characteristic as to deserve more detailed description. The change about to be mentioned affects all the incisors, canines, and first molars of both upper and lower jaws, the bicuspid being scarcely implicated at all; the second molars are also quite healthy. In the first molars the alterations consist of deficiency of enamel on the upper surface of the crown and the presence of spines of uncovered dentine. In the case of the incisors a considerable portion of the crown of each tooth is totally devoid of enamel, and its dentine is also deficient to some extent, so that the teeth are thin, sharp-edged, and of a dirty yellowish color. The transition from the enamel-covered to the diseased part occurs suddenly in a horizontal line at some little distance from the crown of each tooth; the position of this line or step being in each tooth nearly at the same distance from the gum. The general effect when all the teeth are seen together is as if a string had been tied around them when soft and the distal part had withered."]

None of these conditions, however, are in the least degree characteristic of syphilis, the special expression of which in the mouth is to be found only in the permanent upper median incisors. For the recognition and description of the peculiarities of these teeth in the subjects of inherited syphilis we are indebted, as we are for so much else of inestimable value in the study of the disease, to Mr. Hutchinson. In 1863, in a memoir on Syphilitic Diseases of the Eye and Ear, he wrote as follows[125] {294} concerning the symptoms which, in a suspected case, would aid in determining the diagnosis: "By far the most reliable amongst the objective symptoms is the state of the permanent teeth if the patient be of age to show them. Although the temporary teeth often, indeed usually, present some peculiarities in syphilitic children of which a trained observer may avail himself, yet they show nothing which is pathognomonic, and nothing which I dare describe as worthy of general reliance.[126] The central upper incisors of the second set are the test teeth, and the surgeon not thoroughly conversant with the various and very common forms of dental malformation will avoid much risk of error if he restricts his attention to this pair. In syphilitic patients these teeth are usually short and narrow, with a broad vertical notch in their edges and their corners rounded off. Horizontal notches or furrows are often seen, but they, as a rule, have nothing to do with syphilis. If the question be put, Are teeth of the type described pathognomonic of syphilis? I answer unreservedly that when well characterized I believe they are. I have met with many cases in which the type in question was so slightly marked that it served only to suggest suspicion, and by no means to remove doubt; but I have never seen it well characterized without having reason to believe that the inference to which it pointed was well founded."

[Footnote 125: Chapter on "The Means of Recognition of the Subjects of Hereditary Syphilis during the Tertiary Stage," p. 204. Before this, however, he had called attention to the same peculiarities in a paper on "The Means of Recognizing the Subjects of Inherited Syphilis in Adult Life," Medical Times and Gazette, London, Sept. 11, 1858, p. 265.]

[Footnote 126: So far as I know, the only recorded instances by reliable observers of the temporary teeth presenting the peculiar characteristics of syphilis are as follows: In the Transactions of the Odontological Society of Great Britain, vol. ix., 1877, p. 258, Mr. Oakley Coles described a case--without, unfortunately, giving details--in which the "peg-shaped temporary teeth were very characteristic of syphilis." Mr. Coles's abilities are well known, and the fact that at the previous meeting the society had been addressed by Mr. Hutchinson on this very subject would seem to indicate that he was quite familiar with the importance of his statement.

M. Fournier calls attention (Archives de Derm. et Syph., Sept. 25, 1883) to a cast (No. 48) to be found in the collection of M. Parrot in the museum of the Foundling Hospital at Paris. This displays the dentition of a child twenty-seven months old, in which the two upper median incisors are typical Hutchinson teeth. M. Fournier adds that later researches show unmistakably that the temporary teeth may be affected by hereditary syphilis in the same manner as those of the second dentition, although not more than one case of the former is recognized to fifteen or twenty of the latter. He thinks, however, that this proportion would be greatly modified if in the autopsies of young children the alveoli were opened to examine the embryonic teeth. M. Parrot by this means claims to have often discovered lesions of the milk teeth.]

As a matter of course, so positive a statement in a matter of such gravity and importance excited considerable criticism, and the views of Mr. Hutchinson have never been without earnest and often able opponents; but it is safe to say that time has only served to place them on a surer foundation and to enhance their value in the eyes of the profession. That they have undergone no material change in the mind of their distinguished author is shown by his expressions of opinion during the debate on syphilis in the London Pathological Society in 1876,[127] and still later by the following memoranda which he gives as a guide in diagnosis:[128]

"1. No special peculiarities are to be looked for in the first set of teeth.

"2. There can be no more serious blunder than to imagine that bad teeth in proportion to their badness of form are to be suspected of syphilis.

{295} "3. The upper central incisors are the only teeth which are positively characteristic. The others may afford corroborative testimony, but are not to be relied upon alone.

"4. The chief peculiarity is a general dwarfing of the tooth, which is both too short and too narrow, and, from its sides slanting together, presents a tendency to become pointed. This tendency to pointing is always defeated by the cutting off of the end, the truncation being usually effected in a line curved upward, so as to produce a single shallow notch. At the bottom of this notch the enamel is deficient and the dentine exposed, but there is no irregular pitting, as in stomatitis teeth.

"5. The malformations are unusually symmetrical and affect pairs of teeth. The two central incisors resemble each other, and the two laterals are also alike. If any defect passes horizontally across all the incisors at the same level, and affects them all alike, it is probably not due to syphilis.

"6. In syphilis the lateral incisors usually show little or no malformation.

"7. The occurrence of the peculiarities due to syphilis and those due to mercury in the same mouth are exceedingly common."

[Footnote 127: London Lancet, 1876, pp. 56 and 535.]

[Footnote 128: Illustrations of Clinical Surgery, fasciculus xi., London, 1878.]

The great importance of the subject seems to me to justify one more quotation, as showing the opinion at a very recent date of men well qualified to judge of the correctness or inaccuracy of these statements. Mr. C. Macnamara and Dr. Thomas Barlow[129] say: "The characters of the teeth are so valuable when present that it is important to have them clearly noted--the more so that, in spite of Mr. Hutchinson's clear description, they have been much misrepresented. It may be pointed out--(1) That only the upper median permanent incisors are characteristic, and sometimes only one of them is typical, of the disease; (2) that these teeth are generally a little apart, instead of being in apposition, and are more or less dwarfed; (3) that in a typical specimen the width of the cutting edge is narrower than the width of the tooth as it emerges from the gum; (4) that a typical syphilitic tooth presents a single notch, not a serrated margin; and that occasionally, if the notch has not been actually scooped out, there is a little lunula-shaped area which may readily become a notch; (5) finally, that although such teeth, when present, are absolutely pathognomonic, the existence of normal permanent upper median incisors by no means excludes the existence of hereditary syphilis."

[Footnote 129: On behalf of the Collective Investigation Committee, who have issued a circular designed to elicit information as to the effect of syphilis on the civil population of Great Britain. This circular has been sent to physicians, and contains queries as to various points relating to the symptoms of inherited syphilis; among others as to the existence in any given case of "notched, dwarfed upper median incisors," which, with or without other symptoms, would establish the diagnosis of that case. The observations above quoted are explanatory of this question (The British Medical Journal, Dec. 16, 1882).]

I believe this may fairly be taken to represent the general belief at the present day among those best qualified to pronounce upon the merits of the case; and I may say that it is unqualifiedly my own opinion, arrived at after some experience and considerable investigation into the literature of the subject. It is not uncommon, however, to hear doubts expressed as to the value of this sign in the diagnosis of syphilis, and at intervals articles are written or papers read to prove that it is not of the uniform and distinctively conclusive significance that has been attributed to it.[130]

[Footnote 130: "I cannot say more in favor of the diagnostic values of these teeth than that, when {296} present in typical form, they have a certain weight in favor of the existence of hereditary syphilis in the given subject" (Van Harlingen, article "Syphilis" in Internat. Encyc. of Surgery, vol. ii. p. 565). "It has been the custom from time to time since Mr. Hutchinson made his observation to question the validity of his views, both as to the fact of interstitial keratitis being due to hereditary syphilis, and as to the diagnostic values of the so-called characteristic teeth. Thus, it has been asserted, not only in England, but on the Continent, and especially in Germany, that the disease may be the result of malnutrition in scrofulous and rickety subjects; and it has been maintained that the malformation of the teeth is the simple arrest of development in a perverted constitution from other causes than syphilis" (Bumstead and Taylor, op. cit., p. 701). Garretson says (Oral Surgery, p. 316): "Observations will be found to greatly vary concerning the existence of any constancy in phenomenal expressions of the teeth in this relation."

M. Magitot, who has for some years been supposed to pay especial attention to this subject, has recently written an elaborate paper (Gazette des Hôpitaux, Sept. 29, Oct. 4, 11, and 18, 1881) to prove (1st) that dental erosions, as he calls them, are not due to syphilis; and (2d) that they are due to, or are almost invariably associated with, infantile convulsions. He has collected a number of interesting facts, but a very careful study of his article and inspection of his diagrams have convinced me that he does not recognize at all the special peculiarities of the Hutchinson teeth, but includes under his title of "erosion" a variety of widely-differing conditions. He has altogether misinterpreted Mr. Hutchinson's views as stated in his Memoirs on Certain Diseases of the Eye and Ear due to Inherited Syphilis, and represents him as at that time (1863) believing that the cause of the change in the incisors was a mercurial stomatitis. The quotation on p. 294 sufficiently refutes this absurdity. Of course the paper as an argument against the syphilitic origin of these teeth is without the slightest value.]

Corroborative evidence--which, to my mind, is very strong as coming from men whose opportunities for observation of dental peculiarities are almost unlimited--is found in the writings of those gentlemen who have devoted themselves to dental and oral surgery exclusively. Mr. Henry Moon[131] says:[132] "My observations on this subject extend over some twelve years, and include some hundreds of cases; and although, in some details as to the manner of causation, I may differ from the view published by Mr. Hutchinson (before knowledge on tooth-development was advanced as it is at present), yet I must coincide entirely with his general conclusions." "The question really is this: Is there one peculiar conformation of the teeth due to inherited syphilis and not produced by any other cause? The evidence in favor of an affirmative answer to this question appears to me to be so strong that I think the onus of disproof rests with the sceptics."[133] These views were coincided in by the majority of the gentlemen to whom they were addressed, all of them dentists and surgeons of experience and repute, and who included men so well known to the profession as Mr. Oakley Coles, Mr. Samuel Cartwright, Mr. Charles Tomes, and others.

[Footnote 131: Author of the section on "Surgery of the Teeth" incorporated in Bryant's Surgery.]

[Footnote 132: Proceedings of the Odontological Society of Great Britain, vol. ix., 1877, pp. 238, 239. In the same journal for 1875, vol. vii. p. 17, Langdon Down says that whenever he has discovered syphilitic teeth he has "never failed to find confirmatory evidence of the syphilitic history of the case."]

[Footnote 133: He says elsewhere (Bryant's Surgery, 3d Am. ed., p. 429) that the most characteristic change in these teeth is "the lessened breadth of the cutting edge as compared with that of the neck, the vertical groove on their anterior face being often absent, and the notch on their cutting edge not being an absolutely constant feature, and being also subject to obliteration through wear."]

It may be considered as well established, then, that these peculiar teeth--stunted, abnormally narrow at the cutting edge, crescentically rounded with the convexity upward, and the surface inclined upward and forward instead of backward as in normal teeth, widely separated, but converging at their lower edges--are pathognomonic of hereditary syphilis.[134] They are {297} often described as pegged, having been likened to a row of pegs stuck in the gums. This appearance is due to the fact that they are shortened, often projecting not more than half the normal distance from the gum, and are also widely separated; which abnormalities often affect the adjoining teeth as well, and sometimes the entire dentine. It has been asserted that other specific peculiarities are to be found associated with those of the incisors, and Mr. Moon describes as characteristic, and figures[135] small dome-shaped first molars with suppressed angles and absence of enamel from the masticatory surfaces. He believes also that when the upper incisors are typical it is exceedingly rare for the lower incisors to be altogether unaffected.

[Footnote 134: Mr. Hutchinson stated in 1877 that in spite of the fact that many years previously he had challenged any one to bring forward a patient with well-marked syphilitic teeth in whose history no evidence of syphilis could be found, none had come.]

[Footnote 135: Proc. of Odont. Soc. of Great Brit., vol. ix. pp. 241, 242; Bryant's Surgery, Am. ed., p. 429.]

A mistake which I know, from observation, to be frequently made is the confusing of the normal serrations of the cutting edges of recently-erupted normal incisors with the peculiar crescentic edges of the syphilitic teeth. It seems worth while to call especial attention to this, on account of the unpleasant consequences which often follow injudicious questioning based on such supposed syphilitic phenomena. Indeed, the space which has been devoted to this subject of syphilis of the teeth is well warranted, I am sure, by the fact that not only do diagnosis, prognosis, and treatment in cases of great severity, and in both children and parents, often depend upon a recognition of these peculiarities, but that in addition to the clinical and therapeutic problems there are others the solution of which is dependent upon the same knowledge on the part of the practitioner, and which may involve reputation, marital relations, and personal honor and happiness.

The approximate cause of these peculiarities in the incisors can hardly be said to have been demonstrated. Mr. Hutchinson thought at one time[136] that they were due to a stomatitis or an alveolar periostitis, but he has since changed his mind as to that point, believing now[137] that the syphilitic tooth is the result of an arrest of development in the central or first-formed portion of the dentine. The incisors being made up of these lobes or denticles, and dwarfing of the middle one taking place, the two lateral ones fall together. This accounts at once for the small size of the tooth, its shape of an inverted truncated cone, and its crescentic edge.[138] If it were due to stomatitis, it would be more likely to be equally {298} distributed, syphilis in its late manifestations being notably unsymmetrical; there would be no rational explanation of the involvement of one or two teeth while those on either side so frequently escaped; if it were mercurial stomatitis, the enamel too would be involved, as is not usually the case in the syphilitic teeth. It is possible that the central incisors are chiefly affected because they, with the first molars--also affected according to Mr. Moon--and the lower incisors--not infrequently involved--are the first-formed teeth.

[Footnote 136: "The physiognomonical, dental, and other peculiarities by which we recognize the subject of inherited taint when advanced beyond the period of infancy are all of them the direct consequences of special inflammations from which the patient has suffered at former periods; e.g. the synechiæ and lustreless iris of iritis; the malformed teeth of periostitis of the alveolus and dental sacs; the protuberant forehead of hydrocephalus; the flattened nose of snuffles; the pale, earthy, opaque skin of cutaneous inflammation and eruption" (Aphorisms respecting Constitutional Syphilis, 1863).]

[Footnote 137: Proc. of Odont. Soc. of Great Britain, vol. ix., p. 248. See also ibid., pp. 241, 242, remarks of Mr. Moon; also Monthly Review of Dental Surgery, June 15, 1877.]

[Footnote 138: The denticle theory of formation is not necessarily opposed by the fact that there is only a single undivided pulp-cavity in these incisors. Instances of the separate formation of processes of dentinal pulp while others are being used and worn away, all of them finally to unite in a common pulp-chamber, have been observed in lower animals, as in the molar of the elephant.]

The most elaborate article upon syphilitic teeth which has appeared since Mr. Hutchinson's original memoir is one by Fournier,[139] in which, after a very broad and comprehensive consideration of the subject, he arrives at the following conclusions: The hereditary influence of syphilis shows itself in the dental system in two ways, very unequal in point of diagnostic value--viz. first, by a retardation of evolution; second, by the arrest of growth and modifications of structure. The phenomena belonging to the second class may be grouped as follows: First, dental erosion. This is due to imperfect formation of the tooth, the result of a temporary stoppage in its development; but as it produces an appearance like that of worm-eaten wood, it has been called erosion, though in so far as the word conveys the idea of the wearing of a surface which has been previously normal, it is incorrect. The tooth affected with syphilitic erosion has never been normal. The different forms of erosion can be subdivided into groups according as they affect the face or the free edge or grinding surface of the tooth. Of those involving the face there are four types: Erosions en cupule, consisting of small excavations or cups in the surface of the crown; erosions en facettes, in which the surface presents a series of small planes, as though they had been filed; erosions en sillon when there is a linear excavation in the crown of the tooth in the shape of a transverse groove; and erosions en nappe, in which the whole surface is discolored, disorganized, and honeycombed.

[Footnote 139: Archives de Derm. et Syph., Sept. 25, Oct. 2, Oct. 9, 1883. A translation made by the writer may be found in the Dental Cosmos for January and February, 1884.]

A second group of erosions affects the free edge of the tooth, and includes the Hutchinson teeth, with several less important varieties. Dental erosions are multiple, symmetrical, maintain the same level on the crowns of corresponding teeth, and are situated at different heights on the crowns of teeth of different classes. It is evident, therefore, that they are the result of a morbid influence of a general character. There are three theories as to their etiology: (a) that they have no relation to syphilis,[140] but are always connected with infantile eclampsia; (b) that they are exclusively the result of hereditary syphilitic influence; and (c) that they are simply ordinary lesions originating from syphilis with marked frequency, and even in one form--the Hutchinson tooth--appearing to originate only from it. This latter view is the one adopted by Fournier himself. Continuing to group the symptoms due to arrest of growth and modification of structure, we have, second, microdontism, or dwarfing and stunting of the teeth--pegged teeth; third, dental amorphism, in {299} which the teeth are strangely distorted or even transformed in type; fourth, dental vulnerability, or extreme susceptibility to all traumatic or disintegrating influences.

[Footnote 140: M. Magitot, Treatise on the Anomalies of the Dental System, Paris, 1877; Clinical Studies on Erosion of the Teeth considered as a Retrospective Sign of Infantile Convulsions, Paris, 1881; Castanié, Paris, 1879, Thesis No. 384; Rattier, Paris, 1879, Thesis 569; and others.]

Interstitial Keratitis.--The frequency of this form of diffuse inflammation of the cornea, and the diagnostic significance which has been so positively attributed to it--and has been as positively denied--render it of special interest to the general practitioner, who is almost certain to meet with occasional cases, and should be prepared to recognize its possible relation with other, and often graver, conditions.

It begins, commonly, as a slight, diffused haziness situated in the substance of the cornea itself, usually not far from the centre, and at first affecting only one eye. This depends at this stage on the presence of a number of little distinct dots of inflammation, limited to circumscribed, almost microscopic, areas, but later, in a few days, these coalesce, and at the end of a few weeks the whole cornea will probably have become nearly or quite opaque, looking like ground glass. There is no ulceration, and but little congestion as compared with that seen in other inflammatory diseases of the eye, although in the majority of cases there is a fulness of the ciliary vessels and a little photophobia with pains around the orbit. This condition may persist for one or two months, after which the other cornea is nearly always attacked,[141] and is similarly affected, although the disease is apt to pass through its different stages rather faster than in the first eye.

[Footnote 141: In 91 out of 102 cases in Mr. Hutchinson's series--in 6, the left alone; in 5, the right alone (op. cit., p. 123).]

When the height of the disease is reached the corneæ are nearly opaque, a bare perception of light remaining, so that the patient is just aware of the difference between its presence and absolute darkness. Then the cornea which was first involved begins to clear; this is soon followed by improvement in the other one, which in the course of a year or two results in a return to fairly good sight, although in most cases there remain a slight haziness and an abnormal expansion of the cornea.

This favorable result is much influenced by the character of the case, which is sometimes very mild from the outset, and by the thoroughness of the treatment. It is sometimes complicated with iritis, kerato-iritis, cyclitis, posterior choroiditis, secondary glaucoma, etc., in which cases of course the prognosis is most unfavorable. Even in ordinary cases it should be guarded in respect to perfect restoration of function, as clearing of the corneæ may reveal adhesions from iritis or spots of choroiditis disseminata, which could not, of course, have been previously detected. In very mild cases, however, without much evidence of involvement of the other tunics, and which have been submitted to treatment early, it is not uncommon for the cornea to regain almost perfect transparency.

The diagnosis of this condition may generally be made with ease. The ground-glass appearance in the earlier stages and the dull pink or salmon color in the more vascular stage are very characteristic. The vascularity differs from that attending other chronic forms of keratitis, granular lids, etc., in which the vessels are large and superficial, in that in the syphilitic keratitis they are much deeper and very closely interwoven, so that the effect is almost that of an ecchymosis. In other cases both eyes are not so apt to be affected, nor is the tendency to spontaneous cure {300} so marked. The absence of ulceration and the very slight degree of accompanying sclerotic or ciliary congestion are also valuable features.

The conditions which Mr. Hutchinson has known to be most frequently mistaken for it[142] are certain forms of inflammation following small-pox and very superficial ulcers in a healing stage, together with cases of vascular conjunctiva.

[Footnote 142: Op. cit., p. 128.]

There is also said[143] to be some difficulty in distinguishing it from a form of non-syphilitic relapsing cyclitis with corneal opacities and iritis. This, however, is a disease of adults, often limited to one eye, with a strong tendency to relapse, the opacities being more abruptly defined and limited to the region near the circumference.

[Footnote 143: Edward Nettleship in Hill and Cooper, op. cit., p. 267.]

The chief point of interest, however, in the diagnosis of interstitial keratitis is its association with other symptoms of syphilis, upon which, for the general practitioner at least, the diagnosis will usually depend.[144] Mr. Hutchinson's conclusions, drawn from an analysis of 102 cases of interstitial keratitis, bear strongly upon this point, and are as follows:[145] A large proportion of all cases occur in patients between the ages of eight and fifteen, the disease being comparatively rare in early childhood, and still more so after adult age has been reached. He never saw it begin after the age of twenty-six. The patients presented the physiognomical peculiarities of inherited syphilis (see p. 313). In more than half the cases the previous history, especially as regards infancy, was one of hereditary syphilis, and in many instances there was a clear history of infantile syphilis in brothers or sisters. In half the cases no questions were asked as to the existence of venereal disease in the parents prior to the birth of the child. In 29 out of the other half such disease was freely admitted. All the patients had lost in early life nearly half their brothers and sisters. Omitting miscarriages and premature births, it was found that 77 mothers had borne 547 children, of whom only 284 remained alive--an excessive rate of mortality. It appeared in the large proportion of cases in the eldest children in their respective families--a circumstance to be expected in view of the usual behavior of hereditary syphilis in families. Undoubted syphilitic lesions, such as nodes, ulceration of the palate, etc., are not infrequently associated with the keratitis.

[Footnote 144: "To those who have not had opportunities for observation at an ophthalmic hospital I would recommend that the diagnosis should be held to be doubtful if the patient does not present the peculiarities of teeth and physiognomy which I have described, since we find that the latter are the almost invariable concomitants of the true disease" (Mr. Hutchinson, op. cit., p. 128).]

[Footnote 145: Op. cit., pp. 109-129.]

Examination of large numbers of cases has, I believe, established the general accuracy of these statements; and although there is still much difference of opinion as to the exact relation between syphilis and this form of keratitis--whether, for example, it is a symptom of syphilis itself or of a cachexia frequently produced by syphilis, but often by other systemic diseases affecting nutrition--yet, on the whole, so far as I am able to judge of the question outside of its purely technical and ophthalmological relations, I think the weight of modern authority is chiefly on the side of a distinct and practically invariable relation of cause and effect between inherited syphilis and the corneal inflammation.

{301} Mr. James Dixon,[146] for example, proposes to call the disease syphilitic keratitis, and says it is met with exclusively as a sequel of an inherited taint. He adds: "We may meet with some syphilitic keratitis in patients with healthy physiognomy and deformed teeth, or, still more rarely, in those with faultless teeth and the syphilitic cast of features; but to find the true form of keratitis in connection with both good teeth and good complexion is, I think, next to impossible." Many other ophthalmologists express themselves to the same effect more or less strongly. Nettleship,[147] Noyes,[148] Förster,[149] Macnamara,[150] De Wecker,[151] and Carter[152] may be mentioned as having ranged themselves upon this side. On the other hand we have Schweiger,[153] Maunther,[154] Sæmisch,[155] Soelberg Wells,[156] and others who are not convinced that syphilis is the sole nor even, in the opinion of some of them, the principal cause of this disease.

[Footnote 146: Article on "Diseases of the Eye," Holmes's System of Surgery, Am. ed., vol. ii. p. 71.]

[Footnote 147: Op. cit.]

[Footnote 148: Text-book of Ophthalmology.]

[Footnote 149: Handbuch der gesam. Augenheilkunde, vol. vii. p. 186, 1876.]

[Footnote 150: Op. cit.]

[Footnote 151: Ocular Therapeutics, trans. of Forbes, 1879, p. 124.]

[Footnote 152: He even asserts the converse to be true, which is going beyond what I believe can be established in regard to the invariable connection between the two diseases. He says: "The subjects of what we call simply inherited syphilis are liable--nay, are almost sure--to suffer from a peculiar form of interstitial keratitis."]

[Footnote 153: "Hutchinson's view, that this form of keratitis is to be regarded as the direct consequence of congenital syphilis, has not been generally accepted in Germany" (Handbook of Ophthalmology, p. 298).]

[Footnote 154: Zeissl's Jahrbuch der Syphilis, 1875, p. 288.]

[Footnote 155: Graefe and S.'s Handbuch d. Augenheilkunde, 1875, vol. iv. p. 264.]

[Footnote 156: Diseases of the Eye, p. 138.]

Probably the conclusions of Jullien[157] most nearly express the views of those who do not accept in its entirety the syphilitic theory of the disease. He concludes--(1st) that interstitial keratitis coincides frequently, but not invariably, with syphilitic derangement of the dental apparatus; (2) that it is associated with a feeble constitution and with malnutrition, and is thus produced indirectly by scrofula, rheumatism, or syphilis, each of which by its debilitating influence may give rise to a diathesis which favors such morbid developments.

[Footnote 157: Op. cit., p. 1013.]

As a specimen of the evidence which is produced by observers other than Mr. Hutchinson--who, as he himself observes, may be suspected of "that bias which almost necessarily warps more or less the judgment of one who supposes himself to have noted something new"[158]--the statistics of Förster,[159] who apparently takes an unbiassed view of the question, may be noted: In a total of 214 cases of interstitial keratitis evidence of hereditary syphilis other than the corneal disease was noted in 146 (68 per cent.). This number was made up as follows: a, evidence of syphilis in parent, 17; b, evidence of hereditary syphilis in other members of the family, 14; c, evidence in the patient other than keratitis, 115; characteristic teeth, 69; evidences in physiognomy, bones, skin, palate, or choroid, 46. In the remaining 68 cases (32 per cent.), though inheritance of syphilis was not proved, there was, almost without exception, strong suspicion of that disease apart from the keratitis.

[Footnote 158: Op. cit., Preface, p. x.]

[Footnote 159: Graefe and Sæmisch's Handbuch, vii., Part I.]

The condition of the permanent central upper incisors was noted in 138 of the cases; of these they were typical in 73 (53 per cent.); suspicious in 34 (24 per cent.); normal in 31 (23 per cent.). In 15 cases {302} the permanent teeth had not been cut, and in the remaining 61 the state of the teeth was not recorded.[160]

[Footnote 160: Note by Mr. Nettleship, Hill and Cooper, op. cit., pp. 263, 264.]

This seems to me such direct and such unequivocal clinical evidence that it is safe to say, as of the question of syphilitic teeth, that the burden of disproof rests with the doubters, and that we may venture the assertion that interstitial, diffuse, or parenchymatous keratitis is a symptom of inherited syphilis, and that the unmistakable presence of the former disease is sufficient proof of the existence of the latter.

Syphilis of the Nerve-Centres and Nerves.--Until a comparatively recent period our only guide to the course and progress of the nerve diseases of inherited syphilis was to be found in analogy. We knew, for instance, that in acquired syphilis three forms of cerebral disease could be recognized in a general way--one characterized by sudden attack of paralysis, in which the lesion was usually thrombosis from specific endo-arteritis; one in which the symptoms of brain tumor were present, and in which gummata were the cause of the difficulty; and one in which pain, headache, and various functional or convulsive disturbances--chorea, epilepsy, paralysis of single nerves, etc.--were the customary phenomena, and in which periosteal, meningeal, or neuroglial thickenings constituted the pathological basis. The last two are often intermingled both symptomatically and histologically.

Heubner[161] divides cerebral syphilis into three groups, two of which very closely resemble those I have described. In one, however, he includes both the general physical disturbances, incomplete paralysis, and final coma characteristic of tumor and the epileptiform attacks so often due to peripheral or meningeal irritation. This combination is explained by the results of his autopsies, which disclosed in 26 cases in which these symptoms were conjoined a gummous growth in the pia mater of the convexity of one of the cerebral hemispheres, either limited and superficial or involving more or less of the cortex and forming a distinct tumor. The epileptiform attacks were present in 19 out of these 26 cases, while in 20 other cases where the growth was limited to the white substance at the base of the brain they were present only twice. This second form is the apoplectic, followed by general hemiplegia, and depending on disease of the cerebral arteries. His third division is a very ill-defined one, depends much for its limitations upon subjective symptoms, and is of no special interest as applied to the subject of inherited syphilis.

[Footnote 161: Ziemssen, vol. xii.]

Althaus[162] also makes three divisions, two of which are as follows: (1) Cerebral tumor--a gumma either hard or soft. There are then nocturnal headache, sleeplessness, epileptiform attacks, the various phenomena produced by involvement of the cerebral nerves, etc. (2) Disease of the arteries, apoplexy, or softening, followed by hemiplegia.

[Footnote 162: Medical Times and Gazette, Nov. 10, 1877.]

Hutchinson makes a similar division of lesions and symptoms,[163] and the observations of Jaksch, Wilks, and Hughlings Jackson more or less closely coincide with this general classification.

[Footnote 163: Ibid., Feb. 17, 1877.]

Now, in spite of certain striking differences--more apparent than real, however--between inherited and acquired syphilis as regards cause, {303} duration of stages, etc., the essential pathological changes are the same. When syphilis in its later periods attacks the brain or spinal cord or nerve-trunks or vessels of a foetus, it proceeds just as in the adult, the same characteristic accumulation of cells taking place and setting up an arteritis or a meningitis, thickening the sheaths of nerves, or constituting a pericranial node or a gumma according to their number and their situation. We would accordingly expect to find in subjects of inherited syphilis manifestations closely allied to those observed in the adult; and the observations of Barlow,[164] Graefe,[165] Jackson,[166] Heubner,[167] Dowse,[168] and Hutchinson,[169] though comparatively few in number, have already demonstrated the correctness of this supposition.

[Footnote 164: Trans. of Path. Soc. of London, vol. xxviii., 1877.]

[Footnote 165: Archiv für Ophthalmologie, Bd. 1, Ab. i.]

[Footnote 166: Journal of Mental Science, Jan., 1875.]

[Footnote 167: Ziemssen, vol. xii.]

[Footnote 168: Syphilis of the Brain and Spinal Cord, London, 1879, chapter on "Hered. Syph.," p. 67.]

[Footnote 169: Med. Times and Gazette (? Feb. 17, 1877).]

We find, thus, that in these patients meningitis, growths, and arterial disease constitute the three clinical divisions of the disease which have thus far been distinctly differentiated, and the reported cases, with or without autopsy, fall naturally into these classes.

These cases are naturally few, and to make them absolutely reliable it is necessary to have unmistakable evidence of hereditary syphilis in some other form and the demonstration of syphilitic lesions at an autopsy. The case of Dowse, however,[170] includes these requirements. A child twelve years of age, of syphilitic parents, with a history of coryza, sore eyes, and a tubercular syphilide, was attacked with epilepsy, diplopia, facial paralysis, etc., and finally died. At the autopsy three gummatous growths of the surface of the brain were found, and the vessels of the base were found to have undergone the special changes described by Heubner. Their lumen was in some places nearly occluded by an accumulation of spindle-shaped cells between the tunica fenestra and the epithelial lining; and interspersed with them, but particularly in the muscular and adventitious coats, were to be seen enormous quantities of round cells which in many parts seemed actually to replace the normal structures. Dowse's other cases are not at all conclusive in their clinical histories; even the diagnosis was not established by autopsy.

[Footnote 170: Op cit., pp. 71-75.]

Barlow's two cases were both very young children, and are extremely convincing.[171] A child four months old, with snuffles, serpiginous ulcers, etc., and with a syphilitic father, had epileptiform attacks, followed by laryngismus, carpo-pedal contraction, and changes in the choroid. She died aged about fifteen months, and the autopsy disclosed thickening of the pia mater, evidently not tubercular, and changes in the arteries, which in the gradual narrowing of the lumen of the vessel, the absence of ulceration or disintegration or calcification, and the continuity and extent of the cell-proliferation are as different as possible from ordinary atheroma, but correspond precisely with the description of Heubner's cases, which were undoubtedly the subjects of acquired syphilis.

[Footnote 171: Transactions of the Pathological Society of London, 1877, vol. xxviii. pp. 287-291.]

In the second case the symptoms were associated chiefly with the cranial nerves. These were nystagmus, paresis of facial muscles, laryngeal spasms, etc. He died at the age of fifteen months. The vessels of the base were extensively diseased as in the other case, and the fourth, {304} fifth, sixth, seventh, and eighth pairs of nerves were smaller by a new growth of a gummatous nature which had produced almost entire atrophy of the nerve-cylinders. There were cicatrices of the liver and spleen. Cases in which the diagnosis rested upon the history, upon the co-existence of undoubted syphilitic symptoms, and upon improvement under specific treatment are by no means rare.

Berkely Hill reports[172] a typical case of syphilitic epilepsy. A girl aged nine was the first child of the family that had lived, two having previously been born dead. She presented characteristic teeth, traces of choroiditis, and, while under treatment, suffered from both keratitis and iritis. Her first fit was when she was four years of age, and the attacks had recurred frequently since that time. The convulsion was confined to the left side. The left arm was very weak, the weakness having come on gradually, and being especially great in the extensors of the wrist. Under specific treatment the attacks ceased entirely. In this case there was certainly organic disease, probably a gumma on the surface of the right hemisphere.

[Footnote 172: Op. cit., p. 253.]

Other cases reported by the same author, numerous instances of nervous troubles in inherited syphilis reported by Hughlings Jackson,[173] Fournier,[174] Henoch,[175] and Hutchinson,[176] warrant the statement, then, that the nervous diseases of inherited syphilis fall into the same general category as those of the acquired disease; that they may appear at any age, from three or four months to that of puberty; that they depend for their production upon a cell-proliferation which, according to its locality, results in the development of new growths, the production of localized meningeal inflammations, or the obstruction and inflammation of arteries; and that the symptoms are those usually associated with such pathological changes, the diagnosis of syphilis depending chiefly on the history and the presence of other specific phenomena.

[Footnote 173: Journal of Mental Science, Jan. 8, 1875; Transactions of St. Andrew's Med. Graduates' Ass., vol. i., 1868.]

[Footnote 174: Annales des Derm. et Syph.]

[Footnote 175: Nouveau Dict. de Méd. et Chirurgie, p. 885.]

[Footnote 176: Op. cit.]

The prognosis is more unfavorable than in similar cases in acquired syphilis, the meningeal and vascular lesions being the most frequent and showing themselves very obstinate even under careful treatment. Spinal troubles, chorea, and idiocy have been attributed to inherited syphilis, but must occur with great rarity.

Hill[177] reports a case of a child aged five years who had been hemiplegic since he was two and a half years old, and who developed paralysis of the flexors of the ankle in each leg.

[Footnote 177: Op. cit., p. 254.]

Keyes reports a case of a boy five years of age, with nodes on tibiæ and other signs of inherited syphilis, who had two attacks of paraplegia.

Hill reports a case of imbecility associated with inherited syphilis, but there is no evidence that it was other than a coincidence.

Fletcher Beach found not more than 1 per cent. of syphilitic children in the Dareult Asylum, and Mr. Mercier could only trace syphilis in 5 out of 220 female idiots, probably imbecile from birth.[178]

[Footnote 178: Ibid., p. 255.]

Hughlings Jackson only found 1 case of inherited syphilis among 80 {305} cases of chorea. The most carefully recorded cases of the latter affection associated with hereditary syphilis are two reported by Alison.[179]

[Footnote 179: American Journal of the Medical Sciences, July, 1877.]

Syphilis of the Spleen.--Disease of this organ in inherited syphilis is especially important from two points of view. It is a valuable aid to diagnosis, and by its size and the degree of persistence of the swelling gives an approximate indication of the severity of the case.

Attention was first called to the frequency and importance of enlargement of the spleen in early hereditary syphilis by Gee in a paper read before the Royal Medical and Chirurgical Society in 1867.[180] He gave the histories of thirteen children in support of the statement that such enlargement occurred in almost one-fourth of all cases of hereditary syphilis, sometimes with, sometimes without, enlargement of the liver and lymphatic glands. According to him, the degree of splenic enlargement may be taken as a sort of index of the severity of the cachexia; the majority of cases with great enlargement die, but sometimes such children survive, the spleen gradually diminishing in size as the health improves--not diminishing, however, pari passu with such improvement, but remaining for a long time "a monument of past cachexia."

[Footnote 180: British Medical Journal, 1867, vol. i. p. 435.]

Barlow,[181] ten years later, thought Gee had rather understated the proportion of cases in which splenic enlargement occurs, he having found it in 22 out of 28 children with definite hereditary syphilis. Birch-Hirschfeld, Eisenschitz, and Tepel[182] corroborate these observations, finding that the enlargement is almost invariable and that the spleen is often double its normal size.

[Footnote 181: Trans. of Path. Soc. of London, Jan. 20, 1877.]

[Footnote 182: Quoted by Hill and Cooper, op. cit., pp. 164, 165.]

Mr. W. J. Tyson has reported[183] a cure of a child born of syphilitic parents, in whom, at two years of age, the spleen extended downward three and a half inches, reaching the crest of the ilium and approaching closely to the umbilicus. The liver was not enlarged; the urine was not albuminous. He ordered mercury with chalk, one grain every morning and evening, and one grain of iodide of potassium, with ten minims of syrup of iodide of iron to an ounce of water, three times a day. Sixteen months later the spleen had become imperceptible, and three years afterward the child was in excellent health.

[Footnote 183: The Lancet, Oct. 23, 1880.]

The diminution of the liver under treatment appears to take place before there is any diminution in the size of the spleen.[184] This persistence of the latter renders it, as has been stated, a valuable diagnostic sign. In the paper already quoted from,[185] Macnamara and Barlow allude to this as follows: Enlargement of the liver, although it ought to be noted because it is often present in hereditary syphilis, has but little value as a confirmatory symptom--first, because the liver is proportionally large in infancy, and it is difficult to state the limit of what is actually normal; and, secondly, because other causes besides congenital syphilis lead to its enlargement.

[Footnote 184: Barlow, British Medical Journal, Jan. 20, 1877.]

[Footnote 185: British Medical Journal, Dec. 16, 1882.]

With regard to enlargement of the spleen the case is different. Gee's observation, that in the early stage of infantile syphilis some enlargement of the spleen occurs in a large number of cases, has been abundantly {306} confirmed. Although with the subsidence of the other symptoms this enlargement often disappears, so that on post-mortem examination two or three months after there may be no trace of it, yet in a few cases it persists, and indeed sometimes increases, so as to be considerable when the other signs have quite vanished. The importance of this sign is greatest when noted early; as, for example, when the child is from two to three months old, for at that period the enlargement of the spleen due to rickets can hardly come into question.

The condition of the spleen during this period of enlargement seems to be simply that of hyperæmia, or at the most of hyperplasia, Gee's, Barlow's, and Birch-Hirschfeld's autopsies showing no evidence of new growth or of amyloid or other changes.[186]

[Footnote 186: Parrot reports (Le Mouvement méd., Paris, Nov. 23, 1872) two forms of splenic disease produced by inherited syphilis: 1. A simple hypertrophy, which he thinks is secondary to diffuse infiltration of the liver, obstructing the portal circulation and causing the spleen to act as a reservoir; 2. An inflammatory condition resulting in the formation of false membranes around the capsule. His explanation of the first condition is unsatisfactory, because there are numerous cases in which the spleen is enlarged without any involvement of the liver. His other observations have never been confirmed.]

The cause seems to me to be in all probability the well-known effect of syphilis on the glandular system in general, and the lymphatic system in particular, to which I believe the spleen is now generally assigned. The analogy between this slow, persistent, painless enlargement preceding the cutaneous symptoms,[187] unaccompanied by inflammatory symptoms, unattended by any breaking down of tissue, subsiding slowly but evenly under specific treatment, and the behavior of the buboes of acquired syphilis, is certainly very striking.[188]

[Footnote 187: Eisenschitz, Wiener med. Wochenschrift, Nos. 48 and 49.]

[Footnote 188: A similar enlargement occurs, but much more rarely, in the secondary period of acquired syphilis. Weaver noted it in 3 out of 79 soldiers suffering from early syphilis. Wilks and Moxon report cases in which the average weight was 19 oz.]

In most cases of hereditary syphilis there are evidences of disturbance of the gastro-intestinal tract. Vomiting, diarrhoea, colic, anorexia, and emaciation are well-known, but of course not at all characteristic, symptoms.

It has been supposed that the mucous membrane of the entire tract was probably, during the early period at least, and coincidently with the cutaneous eruption, in a condition of hyperæmia and irritation comparable to that of the skin.[189] Whether this be so, or whether it is due to associated involvement of the glandular apparatus, has not yet been determined.

[Footnote 189: "There is surely no a priori probability that a blood disease so severe as syphilis should produce lesions on the skin, in the mouth, and in the eye only--that it should, in fact, affect all the visible parts and avoid all the concealed ones" (Mr. Hutchinson, The Lancet, Feb. 6, 1876).]

Förster[190] has found fibroid degeneration of Peyer's patches in a syphilitic infant who died six days after birth, the glandular structure having been replaced by elevated grayish-red masses of nuclei, cells, and connective-tissue fibre. Ulcers of the intestines have been described, but appear to have had no specific characters.

[Footnote 190: Quoted by Bumstead and Taylor, op. cit., p. 757. His observations were confirmed by Eberth, Roth, and Oser.]

The pancreas has been most extensively studied by Birch-Hirschfeld, who examined seventy-three syphilitic foetuses. In thirteen of them he {307} found enlargement of the pancreas with increased weight and density, proliferation of connective tissue, and in some cases compression--almost obliteration--of the lobules, with atrophy and fatty degeneration of their epithelium.[191]

[Footnote 191: Klebs discovered a gumma in the pancreas of a six-months' foetus.]

The peritoneum has been described by Simpson, Von Baerensprung, and others as occasionally invaded in early hereditary syphilis. There seems to be no evidence that it is ever directly affected, the cases in which death occurs from peritonitis being due usually to trouble connected with the liver or spleen.

Syphilis of the lung, originally described by Depaul and Virchow, has been carefully studied also by Förster, Robin, Lorain, and Cornil, from whose description[192] of the pathology of the condition I condense the following: In the syphilitic foetus born before term, in the syphilitic child born dead at full term, and in the syphilitic children who live a few days, there are found at the autopsy, in the lungs, nodules or tumors, usually superficial, sometimes deep, hard, isolated or in groups, pink, gray, or red in color, with scattered whitish or yellowish points. Their size varies from a pea to a small walnut. They represent a portion of the lung more or less considerable in a state of special lobular hepatization. Sometimes a whole lobe is involved. The affected portion is very dense and covered with thickened inflamed pleura. The lesions are those of chronic pneumonia. The interlobular connective tissue enters into proliferation and presents a large quantity of embryonal cells; the alveolar walls are thick, while the narrow alveoli are livid, and even filled by epithelial cells, which are of the pavement form in contact with the walls, round in the centre of the alveoli. As the process progresses the epithelial cells become fatty, degenerated, and subsequently broken down and absorbed, while the embryonal interalveolar tissue rapidly organizes into fibrous tissue. Thus results a small fibrous tumor, in which a gumma may ultimately develop.[193]

[Footnote 192: Syphilis, trans. of Simes and White, Philada., 1882, p. 404.]

[Footnote 193: Cornil and Ranvier's Path. Histology, Am. ed., 1880.]

It is impossible to confound this syphilitic pneumonia with tuberculosis. The granulations of tubercle are never congenital.

The fact that in syphilitic interstitial hyperplasia the change begins in the interlobular connective tissue and around the interlobular vessels, at first consisting of small spindle-shaped and roundish cells which quickly develop into connective tissue, and the fact that blood-vessels are freely produced among the fibres of this new tissue, seemed, in the estimation of those pathologists who took part in the discussion on visceral syphilis in 1877, to constitute its most distinctive feature.[194]

[Footnote 194: Trans. of Lond. Path. Soc., vol. xxviii.--views of Green, Jones, Greenfield, Moxon, and others.]

Gummata in the lungs of children suffering from inherited syphilis have been described by a number of writers. They appear likewise to begin in the walls of the blood-vessels or the bronchioles. They differ from tubercular nodules in being few in number--not more than half a dozen usually--and are generally confined to one lung.

The condition of the arteries in the few cases in which they have been noticed as affected by inherited syphilis was precisely similar to that found by Heubner in the arteries of adults.[195]

[Footnote 195: See Cornil, op. cit., p. 305.]

{308} The symptoms are very variable; new-born children often die asphyxiated in a few days. If they live longer, the disease develops into a more generalized broncho-pneumonia.

Syphilis of the Larynx.--The hoarse cry of the new-born infant so characteristic of hereditary syphilis depends upon the presence of hyperæmia, of mucous patches, or even of extensive ulceration. I am inclined to think that the first is the more common, as if it were otherwise cases of death from oedema glottidis or other forms of laryngeal obstruction would be oftener met with. When ulceration does exist it is generally, but not invariably, secondary to pharyngeal ulcers.[196]

[Footnote 196: See synopsis of six cases of George M. Lefferts, reported in Bumstead and Taylor, op. cit., p. 754.]

Bronchial catarrh, giving rise to cough, and sometimes to considerable embarrassment of respiration, is a not infrequent complication of laryngeal syphilis.[197]

[Footnote 197: Schnitzler, Die Lungen Syphilis, etc., 1880, S. 41.]

Later troubles of the larynx in connection with inherited syphilis have not yet been carefully enough studied to warrant us in drawing any distinction between them and the usual symptoms seen in the acquired disease.

Syphilis of the testicles has been studied by Henoch,[198] Cornil,[199] Parrot,[200] Hutinel,[201] North,[202] Bryant,[203] and others. It is found to consist of a true interstitial orchitis, very closely resembling that seen in the syphilitic testicles of adults. Hutinel's investigations, based on ten cases, showed the testicles slightly enlarged and harder than normal, the scrotum pendent, the epididymis normal, the tension of the tunica vaginalis and tunica albuginea slight. The basis of the lesion is in a collection of small round embryonal cells resembling lymph-cells, arranged in the connective tissue around the arterioles which come from the tunica albuginea. This may be accompanied by a more or less marked diffused interstitial orchitis, or there may be only a thickening from the new formation of small round cells on the connective tissues of the testicles. Cornil found the seminal ducts separated by numerous round or fusiform cells. The disease usually occurs at from two months to three years of age; both testicles are generally involved, and are enlarged, hard, inelastic, and frequently nodulated. Mercurial treatment generally causes a marked improvement unless the inflammation has already resulted in the development of a new fibroid formation, in which case it would be likely to remain unaffected by any form of treatment. Inunctions with diluted mercurial ointment, iodoform, etc. are useful adjuvants.

[Footnote 198: Schmidt's Jahrbuch, 178, No. 4.]

[Footnote 199: Op. cit., p. 420.]

[Footnote 200: Rev. mens. de Méd. et de Chir., Paris, Feb., 1878.]

[Footnote 201: Ibid.]

[Footnote 202: Med. Times and Gaz., Lond., 1862, vol. i. p. 403.]

[Footnote 203: Ibid., Dec., 1863.]

The kidneys are not infrequently involved in inherited syphilis. Parrot reports the pathological change to consist of a proliferation of small round cells in the intertubular connective tissue, followed by contraction, obliteration of the tubules, and degeneration of their epithelium.

Bradley has reported[204] the case of a child aged four months in whom a well-marked syphilitic eruption and an attack of acute Bright's disease were coincident. Mercurial treatment for three weeks cured both.

[Footnote 204: British Med. Journ., Feb. 4, 1876.]

Coupland has reported two cases of parenchymatous nephritis associated with inherited syphilis, but advances no proof that it was not an accident. {309} Gummata have been from time to time found in the kidneys of very young children who have died from their effects and from other visceral lesions due to syphilis.[205] Cases of enlargement, of fibroid, fatty, and gelatinous degenerations of the suprarenal capsules, have been recorded.

[Footnote 205: See discussion in Clinical Soc. of London, Jan., 1880; "Remarks on Visceral, and especially on Renal, Syphilis," by Barthelémy, Annales de Derm. et Syph., April, 1881.]

The thymus gland is occasionally found in syphilis to have undergone alterations claimed by Dubois, Depaul, and others to be syphilitic in their nature, but ascribed by Parrot simply to degenerative changes due to malnutrition. The gland does not appear to undergo any marked alteration in size, color, or consistency, but is found after death to contain a small quantity of purulent matter.

The tendency of syphilis is certainly not, as a rule, to the formation of pus. Nearly all the lesions we have studied with the exception of breaking-down gummata have consisted in various forms of cell-proliferation or accumulation, and not in the formation of abscesses, and it is not probable that this is an exception. I doubt very much the syphilitic character of these changes.[206]

[Footnote 206: Lancereaux believed that it was due to the breaking down of a gummy deposit, but that seems to be entirely hypothetical, none having been discovered. Weisflag (quoted by Bumstead) arrives at the following conclusions after studying the lesion and the literature of the subject: 1. This thymus abscess does exist. 2. When associated with other signs of congenital syphilis it indicates that the father or mother of an infant suffers or has suffered from syphilis. 3. It is possible, but not proved, that this affection may exist in children in whom there are no symptoms of syphilis, but its existence renders the diagnosis of hereditary syphilis probable, even if the disease of the parent is not proved. 4. Such is the great similarity in the appearance of pus and of the secretion of the thymus that they cannot always be distinguished.]

THE DIAGNOSIS AND PROGNOSIS OF INHERITED SYPHILIS.--In reviewing the general course of a case of inherited syphilis it seems evident that the differences between it and the acquired disease which have been so much dwelt upon are apparent rather than real.[207] The primary stage is of course missing, and on any theory of the essential nature of syphilis this is readily comprehensible. Whether the chancre is the first symptom of a constitutional disease, or, as I believe to be the case, is the simple accumulation at the point of original inoculation of the cells which constitute the syphilitic virus--or are at any rate its carriers--it would naturally be in the first case undiscoverable, in the second nonexistent.

[Footnote 207: "That the noteworthy differences between chancre-syphilis and the inherited disease are to be interpreted by considerations of the tissues of the growing child and the adult, is made very probable by what is observed when a mother near the end of pregnancy becomes infected with primary disease. In such a case the foetus nearly full grown acquires the disease, without a chancre, directly from the maternal blood. It is acquisition, not inheritance, for at the date of conception both the paternal and maternal elements were free from taint, and during the first six, seven, or even eight months of intra-uterine life the foetus remained healthy. Yet, as I have proved elsewhere by citation of cases, syphilis obtained in this peculiar method resembles exactly that which comes by true inheritance, and not that which follows a chancre. This important fact goes, with many others, in support of the belief that the poison of syphilis remains identical, however obtained, and that the differences which are so patent in its manifestations are due to differences in the state of its recipient" (Mr. Hutchinson, article on "Transmission of Syphilis," Brit. and For. Med.-Chir. Rev., Oct., 1877, p. 475).

"It is not true that the diversity of symptoms presented by infants authorizes us to admit a congenital and an hereditary syphilis. Whatever the mode of infection, it is impossible to make this distinction" (Ricord, note to John Hunter's Works, 1883).]

The secondary stage, characterized in the acquired form chiefly by {310} lymphatic engorgement and symmetrical, widely-spread, polymorphic cutaneous and mucous eruptions, and pathologically by a marked tendency to the proliferation of certain new small round nucleated cells, upon the presence of which depend all the manifestations of the disease, is in inherited syphilis strictly analogous. Eruptions of the same character make their appearance, differing only in minor points, as in a greater tendency to become moist or ulcerated, due to the more delicate texture of the infantile epidermis. To the same cause must be assigned the macroscopic peculiarities of the only syphiloderm said to be peculiar to infantile syphilis--pemphigus--which has been shown, however, to have a papular basis, and in that way to conform to all the other secondary eruptions.

The lymphatic engorgement either exists in the infant as in the adult or has its analogue in the enlargement of the spleen and liver--especially the former, which is almost as constant a phenomenon as is general glandular enlargement in acquired syphilis. The same pathological changes occur, the same infiltration of cells producing, according to their situation, papular, pustular, or mucous patches, or inflammation of such structures as the iris, choroid, or retina.

The tertiary stage, except in the fact that its phenomena may appear unusually early and may be commingled with those of the secondary period,[208] does not widely differ in the hereditary from that of the acquired disease. It affects the same tissues, results in the same pathological formations, and is preceded by the same period of latency or quiescence of variable duration. There is no reliable evidence with which I am familiar to show that in this stage inherited syphilis is either contagious or transmissible--another point of close resemblance between the two varieties under consideration.

[Footnote 208: This is by no means unknown even in the acquired form; frequent examples of it have been recorded, and it can be readily explained either on the theory of relapses in parts previously diseased (Hutchinson), or on that of obliteration of lymphatic trunks and accumulation of nutritive waste (Otis).]

In considering the question of diagnosis, therefore, we have an excellent guide in the fact that the disease conforms in most respects to the general laws of acquired syphilis, and that our knowledge of the latter affection will be a valuable aid to recognition of the former.

The chief elements of diagnosis and prognosis of inherited syphilis in its various stages may then be summarized as follows:

A history of syphilis in either parent is important just in proportion to the shortness of the interval between the time of infection and the date of conception. In other words, the shorter that interval the more likely (a) that the child will have syphilis, (b) that it will have it in a severe or fatal form. If the mother has been syphilitic and the father healthy--which is rare--it is perhaps more likely that the child will be diseased than when the reverse is the case. If both parents were syphilitic at or before the time of conception, the probability that the disease will be transmitted, and in a severe form, is much increased. There is no evidence to show that inheritance from one parent results in a graver variety of the disease than when it is derived from the other.

A history of abortion or miscarriage on the part of the mother should have weight in the determination of any given case, and if such accidents {311} have been very frequent their diagnostic importance is greatly increased. The loss of elder brothers or sisters and the causes of death, with the precedent symptoms, should be carefully inquired into. The nearer either of these occurrences--abortion or death of elder children, if there is a fair presumption that they were due to syphilis--has been to the birth of the patient in question, the greater the likelihood that the latter has been infected.

Upon examining the product of abortion or stillbirth the most easily observable symptoms will be those of the skin. Maceration and elevation of the epidermis into bullæ are in themselves hardly characteristic, though they may--especially the latter--be regarded as suspicious. If the cutaneous lesions are, however, distinctly papular or pustular or ulcerative, or if the bullæ have all the characteristics of syphilitic pemphigus, the diagnosis is assured.[209]

[Footnote 209: "It is probable that very early abortions are less rare than statistics indicate, but are often unsuspected."

"It is impossible to demonstrate the existence of syphilitic lesions in foetuses expelled during the first months of pregnancy. Later, the signs which have the greatest value are the lesions of the epiphyses of the long bones. When the foetus has nearly arrived at full term, and is not macerated, visceral and cutaneous lesions may be observed. According to Mewis, the skin eruptions cannot be seen before the eighth month, and are only recognizable on foetuses whose death has been very recent or who are born living. Pulmonary lesions may be determined at the end of the sixth month. Those of the pancreas are met with in about half the foetuses which perish a little before or a little after birth. The lesions of the liver, the spleen, and the bones may be recognized even in macerated foetuses, this frequency increasing from month to month" (Nouv. Dict. de Méd. et Chir., vol. xxxiv. p. 864).]

The most distinctive symptom--one which may really be considered as pathognomonic, is, however, the inflammation of the diaphyso-epiphysial articulations, with or without their disjunction. Distinct enlargement of the spleen or liver, and arachnitis with hydrocephalus, are valuable diagnostic points, and the presence of gummata--not very infrequent--would of course be conclusive.

At birth the syphilitic child may be small, stunted, emaciated, weazened, senile in appearance; this would properly give rise to suspicion, but may be associated with any disorder of nutrition on the part of child or mother. It may also disclose cutaneous or mucous eruptions evidently specific in character. The most common of these at this early date is the bullous eruption affecting the palms and soles, sometimes distributed over the whole body, and, as it indicates a feeble resistance of the tissues to the tendency to exudation and cell-growth, is usually a precursor of an early and fatal termination. In any event, marked symptoms at time of birth render the prognosis highly unfavorable.

It is quite as common, however--perhaps more so--for the subject of hereditary syphilis to give no evidence of the disease at birth, but even to appear healthy and well-nourished. In such cases the first symptoms of the disease appear, on an average, in from six weeks to two or three months, and consist principally of coryza (snuffles), hoarseness of voice, and syphilodermata. The latter may be macular, papular, pustular, or bullous. They are usually polymorphous, irregular in shape, dark coppery-red in color, with sometimes a glazed or crusted, but oftener a moist or ulcerating, surface, with a strong tendency to coalesce into large patches, or to form irregular serpiginous ulcers, or to take on hypertrophic growth {312} and develop into condylomata. Eruptions which are squamous and are situated about the mouth and chin and on the body, the legs, or the soles of the feet, though exceptional, are of more value than those on the nates, where the results of irritation from urine and feces may closely simulate syphilodermata.

Mucous patches on the tongue, cheeks, tonsils, and pharynx are common, often extending to the larynx, increasing the hoarseness, and to the nasal cavities, aggravating the snuffles. Both of these occurrences, by interfering with the respiration of the child and rendering its nursing interrupted and insufficient, greatly add to the gravity of the case. Enlargement of the spleen (common), enlargement of the liver (less so), and iritis (rare), may be mentioned among the phenomena of this stage, often associated with the skin eruptions.

About the time of the subsidence of the rash there may be developed the specific inflammation at the junction of epiphyses and diaphyses which produces a swelling of the long bones near their ends. The child will be noticed to cry a little when, for example, the wrist or elbow on one side is washed, and not to use these joints as much as the corresponding ones on the other side. The parts are not hot, only slightly tender, and as yet there is but little swelling. Later, the droop and the disuse of the affected limb become more noticeable and simulate infantile paralysis. There is, however, no wasting, no alteration of reaction by faradism, no real loss of power, so that the term pseudo-paralysis is an appropriate one. In a week or two similar symptoms will occur in the bone on the opposite side, and finally the ends of all the long bones may be affected; ordinarily the elbows, wrists, knees, and shoulders are the joints involved. Suppuration is rare, disjunction of the epiphysis from the diaphysis common. Recovery is apt to take place spontaneously within a month. The associated changes are chiefly endosteal at the junction of the shaft with the epiphysis, but there is also a little periostitis or perichondritis, which is the principal cause of the external swelling. Moderate deformity may ensue.[210]

[Footnote 210: For the diagnosis from rickets see p. 290.]

Similar changes occurring in the cranial bones give rise to what has been called the natiform skull. During the first year it is very common for syphilitic children to develop a number of lenticular swellings on the cranium, which appear symmetrically around the anterior fontanel, but at a little distance from it; i.e. one on each frontal and one on each parietal bone. These are said to be "bossed." They are at first circumscribed, and in a child nine or ten months old often measure three-quarters of an inch to an inch in diameter. They are at first circular, afterward more irregular, and finally tend to organize, becoming diffused and massive and causing a permanent thickening of the skull.

These symptoms which have been described are the prominent ones occurring during the first six or eight or twelve months of life. If they do not manifest themselves before the eighth month, it is highly probable, even in a case with a syphilitic parental history, that the child will either escape altogether or that the secondary stage has been very slight and altogether intra-uterine and unattended with noticeable phenomena. If during this first year the child's cachexia is marked, if there are any intercurrent diseases, if the symptoms show themselves early, if the nasal or laryngeal affection is severe, if the eruptions are markedly bullar or {313} pustular or ulcerative, if the enlargement of the spleen is great or the osseous lesions precocious or grave, and if, especially, there is any intermingling of tertiary symptoms, gummata, nodes, etc.,--the prognosis will be unfavorable.

From adolescence on through adult life the diagnosis of inherited syphilis will depend on the following points: First, of course, the history of parental or of infantile syphilis, or of both. Then a group of physical and physiognomical peculiarities, which are not definitely characteristic, and are of little value when taken separately, but are of considerable importance when all or a majority are present in any given case. These are low stature or puny development proportionate to the severity of the intra-uterine and infantile symptoms; a pasty, leaden, or earthy complexion,[211] a relic of previous syphilodermata, probably also a result of malnutrition; a prominent forehead, bulging in the middle line at and within the frontal eminence, and due either to thickening of the skull or to a previous arachnitis and hydrocephalus before the ossification of the fontanels; a flat, sunken bridge to the nose, due to the coryza of infancy extending to the periosteum of the delicate nasal bones, and either interfering with their nutrition or partially destroying them; dryness and thinness of the hair, with brittleness and splitting of the nails; synechiæ and dulness of the iris (rare); ulcerations of the hard palate;[212] and periosteal thickenings or enlargements of the shafts of the long bones near the ends, or slight angular deformity, results of the osteo-chondritis of infancy.

[Footnote 211: Trousseau (Clinical Lectures, vol. ii. p. 588, Philada., 1873), after calling attention to this peculiar hue of the face, says: "It not unfrequently happens that the physician, taught by long familiarity with this appearance, will almost at once diagnose syphilis after having simply seen the child's face, although the peculiar hue can be but vaguely described in words. The visage presents a special shade of bistre; it looks as if it had been lightly smeared with coffee-grounds or a very dilute aqueous solution of soot. There is neither the pallor, the icteric hue, nor the straw-yellow tinge of skin seen in other cachectic affections; the tinge is not nearly so deep, but is almost like that of the countenance of a recently-delivered woman, and either does not extend at all, or only partially, to the rest of the body. I know no disease except syphilis in which a child's skin has this peculiar color; and consequently, when it is well marked, it has more diagnostic value than any other symptom."]

[Footnote 212: Mr. Oakley Coles reports (Proc. of Path. Society of Great Britain, vol. vii. p. 5) several cases of inherited syphilis in which there was wide separation of the jaws in the median line. In one family one member had typical teeth and wide separation; three others had the same separation, but not the characteristic teeth. It was suggested that in such cases the teeth were in size far below the average, and that the condition was that often observed where the jaws are in development in excess of the teeth which they contain. I. E. Atkinson details some interesting cases of this lesion in late hereditary syphilis, and attributes to it considerable diagnostic importance (American Journal of the Medical Sciences, New Series, vol. lxxvii., Jan., 1879, p. 71).]

A much more valuable group of symptoms, however, are the following, which are mentioned in the order of their importance, any one of the first three being almost or quite conclusive:

Dwarfed permanent median upper incisors, broader at the top than at the cutting edge, which is crescentically notched, separated by an undue interval and converging toward each other.

Evidence of past or present interstitial keratitis--a dusky and thin sclerotic in the ciliary region and slight clouds here and there in the corneal substance, there being no scars on its surface--or of disseminated choroiditis; patches of absorption especially around the periphery.

{314} A radiating series of narrow cicatricial scars extending right across the mucous membrane of the lips, or a network of linear cicatrices on the upper lip and around the nostrils, as well as at the corners of the mouth and on the lower lip.

Periosteal nodes on one or many of the long bones; sudden, symmetrical, and complete deafness, without otorrhoea and unattended by pain or other subjective symptoms.[213]

[Footnote 213: In a few instances there has been noticed an arrest of sexual development; in one case of Hughlings Jackson's there was such an entire absence of all sexual characteristics that it was supposed that the ovaries had been destroyed by syphilitic inflammation in early life.]

Late or tardy hereditary syphilis is rarely dangerous to life. The prognosis is almost unvaryingly favorable unless some grave visceral complication, such as interstitial pneumonia, gummata of the brain, liver, or kidney, or meningeal and periosteal inflammation within the cranium, should occur.

TREATMENT.--The prophylactic treatment, or that directed to the health and sexual relations of the parents previous to conception, has already been sufficiently considered. That of the mother during pregnancy, after having conceived from a syphilitic husband, or having had antecedent syphilis, or having contracted it by direct contagion subsequent to impregnation, is simply that of acquired syphilis in either adult or child. Mercury in its full physiological dose is the drug indicated. It may not be amiss to combine with it iodide of potassium in moderate doses, but the practice of employing the latter to the exclusion of the former is both theoretically and clinically unsound. Care should especially be taken to give it in such a manner, either by inunction or vaporization or so guarded with opium, that it will not produce any irritating effect on the intestinal canal, the sympathy between which and the uterus may, in the event of a strong purgative action being set up, lead to an abortion.[214]

[Footnote 214: "In respect to prophylaxis as applied to infants, all chances of infection should be entirely removed whenever constitutional symptoms exist or the nature of the primary symptoms renders them probable. Our caution should be carried still farther, and in the absence of all appreciable symptoms we should assure ourselves by the antecedents, so far as possible, that the parents are not under the influence of a syphilitic diathesis; in which case they may give birth to infected infants until appropriate treatment shields the latter from infection. With still stronger reasons, when the mother during pregnancy is affected with primary syphilitic symptoms of such a character as to give rise to secondary symptoms, or if the latter already exist, we should hasten to cope with them, and, far from regarding pregnancy as a contra-indication to treatment, should recollect that it generally prevents the disease in the infant, and when skilfully administered obviates the frequent abortions which syphilis excites. When primary symptoms have been contracted by the mother a short time before delivery, since the infant may be infected in its passage into the world, the same course should be followed with it as with a person who has just exposed himself to an impure connection" (Ricord, note on prophylaxis of venereal disease appended to his edition of John Hunter's Treatise on Venereal, Philada., 1853, p. 481).]

As we have seen that the pathology, the stages, and the general course of hereditary syphilis are all closely related to or identical with the same phenomena in the acquired disease, and so know that they both depend upon the same ultimate cause, whatever that may be--a virus, a fungus, or a degraded cell--it follows that the same principles should govern us in the treatment of the one as in that of the other.

We know from clinical experience that mercury exercises an almost {315} controlling influence over the secondary manifestations of acquired syphilis, whether by acting as a true antidote or as a tonic, or by virtue of its property of hastening destructive metamorphosis and thereby facilitating the absorption or elimination of new cell-growths. We know also that iodide of potassium, probably by virtue of its powerful stimulating influence on the lymphatic system, has an equal power over the tertiary growths, which by their pressure upon or situation in important tissues or organs may be so destructive. There is no reason, therefore, by analogy why these drugs should not, comparatively speaking, be equally beneficial in hereditary syphilis; and such is, indeed, found to be the case. In the latter affection, however, there are two elements which should modify the treatment somewhat, and must be taken into consideration. These are--1st. The existence of a more or less profound cachexia influencing all the nutritive and formative processes, and in itself, entirely apart from any definite specific involvement of vital organs, threatening life. 2d. The not infrequent occurrence during the secondary period of symptoms--notably gummata--belonging to the tertiary stage.

The first indication is met by making the treatment from first to last not only antisyphilitic, but also supporting or even stimulating; and with this object in view especial attention should be paid to nutrition. It may be stated, axiomatically, that for every reason, whenever it is within the bounds of possibility, the nurse of a syphilitic child should be its mother. To her it is harmless--to every other woman, not already syphilized, it is in the highest degree dangerous. Space will not permit me here to discuss the medico-legal aspect of the interesting question as to relations between such children and the outside world, especially as represented in their nurses. It will suffice to say that it is criminal and legally punishable to induce any healthy woman to act as wet-nurse to a syphilitic child unless she does so with a full knowledge of the risks she runs in undertaking that function. In the rare cases where with such information she still consents to suckle the child a written statement of the facts of the case should be signed by her, with the proper legal formalities, for the protection of the physician and the family.

If the mother has died or on account of ill-health is unable to nurse her child, and if no wet-nurse willing to enter the above agreement can be obtained, the possibility and propriety of obtaining one who has already had syphilis must next be considered. This idea to many parents seems revolting, but will naturally be less so to those who have themselves had the disease, and is, besides, so almost vitally important to the child that no hesitation should be felt about making the suggestion. If it is accepted, and if there is any opportunity for making a selection, it may be said that the more robust the present condition of such a nurse, and the more remote the date of her syphilis, the better will be the chances of the child.

If neither mother nor wet-nurse can be had to suckle the child, it must be fed by cow's, goat's, or ass's milk or by artificial alimentation; but its prospect of life will be greatly, immeasurably, reduced. In addition to careful feeding a little careful tonic treatment should from the first be employed in conjunction with the specific remedies, iodide of iron, cod-liver oil, and preparations of the phosphates being the most useful drugs.

The existence of the second condition, which, as I have stated, exercises a modifying influence upon treatment--the early appearance of tertiary {316} symptoms--is probably due in many cases at least to an overwhelming of the lymphatic system by the new cell-growth, which not only greatly increases the amount of material to be transported by the lymphatics, but at the same time, by invading their walls and diminishing their lumen, greatly cripples them. Accumulations of nutritive matter and of these new cells then take place, forming the characteristic new growths or deposits which we call gummata. This leads us to combine with the mercury from the beginning, at least in all cases where bony or periosteal involvement, suppuration, or the existence of gummata points to this condition, small doses of iodide of potassium or of some other soluble and easily decomposed iodine salt.

The principle of treatment being thus recognized, the routine procedure may be thus described: Give mercury as soon as the diagnosis of syphilis is assured--preferably by inunction. Sir Benjamin Brodie's opinion, expressed many years ago, still represents that of the profession:[215] "I have tried different ways of treating such cases. I have given the child gray powder internally and given mercury to the wet-nurse. But mercury exhibited to a child by the mouth generally gripes and purges, seldom doing any good, and given to the wet-nurse it does not answer very well, and certainly is a very cruel practice.[216] The mode in which I have treated cases for some years past has been this: I have spread mercurial ointment, made in the proportion of a drachm to an ounce, over a flannel roller and bound it around the child once a day. The child kicks about, and, the cuticle being thin, the mercury is absorbed. It does not either gripe or purge, nor does it make the gums sore, but it cures the disease. I have adopted this practice in a great many cases with signal success. Very few children recover in whom mercury is given internally, but I have not seen a case where this method of treatment has failed."

[Footnote 215: Clinical Lectures on Surgery, Philada., 1846, p. 230.]

[Footnote 216: This, the so-called indirect method, is altogether unreliable, and should only be employed as a forlorn hope in those cases where in every other way mercury sets up gastro-intestinal irritation.]

When, for any reason, as irritation of the skin, this cannot be employed, probably the best form of giving mercury by the mouth is in the following formula:

Rx. Hydrarg. cum Creta, gr. j to vj. Sacch. alb. gr. xij. In M. ft. chart No. xij.

S. One powder three times a day, to be taken soon after nursing.

Iodide of potassium may be given separately in a syrupy solution in doses of a half-grain to a grain, or if there are any marked tertiary symptoms even in much larger doses, three or four times daily.[217] {317} Treatment should, of course, be continued long after the disappearance of syphilitic symptoms, and it would probably be well to continue the mixed treatment intermittently until after puberty.

[Footnote 217: Wm. Campbell of Edinburgh was in the habit of commencing with doses of a quarter of a grain of calomel and two grains of creta præparata, once daily for the first ten days. He afterward progressively increased the calomel to a quarter of a grain twice each day. Sir John Rose Cormack says (Clinical Studies, vol. ii. pp. 423, 424, London, 1876) that an infant six weeks old will generally bear these doses well. In cases where they do not, he was in the habit of ordering a solution of half a grain of the bichloride in three ounces of distilled water and one ounce of syrup--one to two teaspoonfuls every six, eight, or twelve hours. When he used mercurial "swabbing" he employed from one to four drachms of unguent, hydrargyri to the ounce of lard. He alternated this treatment with short courses of the syrup of the iodide of iron, and continued the treatment up to the period of dentition. He says he has generally obtained excellent results by these methods.]

With the treatment of special symptoms the general practitioner has little concern. The cases of visceral syphilis in very young children are generally fatal. Those that recover do so in response to the active use of the above remedies. Later, the prognosis is more favorable, the treatment the same. Of course moist eruptions should be dusted with some astringent or absorbent powder; mucous patches should be cauterized; and great attention should be paid to avoidance of sources of cutaneous irritation--frequent changing of diapers, etc.--but the general methods are the same as in the adult.

{319}

DISEASES OF THE DIGESTIVE SYSTEM.

DISEASES OF THE MOUTH AND TONGUE. DISEASES OF THE TONSILS. DISEASES OF THE PHARYNX. DISEASES OF THE OESOPHAGUS. FUNCTIONAL AND INFLAMMATORY DISEASES OF THE STOMACH. SIMPLE ULCER OF THE STOMACH. CANCER OF THE STOMACH. HEMORRHAGE FROM THE STOMACH. DILATATION OF THE STOMACH. MINOR ORGANIC AFFECTIONS OF THE STOMACH. INTESTINAL INDIGESTION. CONSTIPATION. ENTERALGIA (INTESTINAL COLIC). ACUTE INTESTINAL CATARRH (DUODENITIS, JEJUNITIS, ILEITIS, COLITIS, PROCTITIS). CHRONIC INTESTINAL CATARRH. CHOLERA MORBUS. INTESTINAL AFFECTIONS OF CHILDREN IN HOT WEATHER. PSEUDO-MEMBRANOUS ENTERITIS. DYSENTERY. TYPHLITIS, PERITYPHLITIS, AND PARATYPHLITIS. INTESTINAL ULCER. HEMORRHAGE OF THE BOWELS. INTESTINAL OBSTRUCTION. CANCER AND LARDACEOUS DEGENERATION OF THE INTESTINES. DISEASES OF THE RECTUM AND ANUS. INTESTINAL WORMS. DISEASES OF THE LIVER. DISEASES OF THE PANCREAS. PERITONITIS. DISEASES OF THE ABDOMINAL GLANDS (TABES MESENTERICA).

{321}

DISEASES OF THE MOUTH AND TONGUE.

BY J. SOLIS COHEN, M.D.

Stomatitis.

DEFINITION.--Inflammation of the interior of the mouth.

The term Stomatitis is used to designate inflammatory affections of the mucous membranes of the structures of the interior of the mouth, including thus the mucous membrane of the lips, gums, tongue, cheek, palate, and anatomical adnexes. Inflammatory affections of the mucous membrane of the palate, palatine folds, and tonsils are usually described more particularly under the heads of angina, sore throat, and tonsillitis.

Stomatitis occurs idiopathically, deuteropathically, and traumatically.

Several varieties of stomatitis occur, sufficiently characteristic to require separate description: viz. erythematous or catarrhal, aphthous or vesicular, folliculous or glandular, pseudo-membranous or diphtheritic, ulcerous, gangrenous, cryptogamous or parasitic, and toxic.

Stomatitis Catarrhalis.

Simple, superficial, erythematous, or catarrhal stomatitis; pultaceous stomatitis.

DEFINITION.--A simple inflammation or erythema, general or partial, of the mucous membrane of the interior of the mouth.

It occurs both in adults and in children, and may be primary or secondary, acute or chronic. In adults and adolescents it accompanies catarrhal and ulcerous affections of the throat, and is described, therefore, to a certain extent, in connection with these affections.

SYNONYMS.--Ordinary or common diffuse Inflammation of the mouth; Erythema of the mouth; Oral catarrh.

ETIOLOGY.--In many cases of catarrhal stomatitis, both in adults and in children, the affection is of obscure origin and the cause eludes detection. In the great majority of instances the cause lies in some irritation of the alimentary tract, whether local or at a distance.

The local causes, which are by far the more frequent, include every variety of topical irritation to which the oral mucous membrane is in itself liable or to which it may be subjected. Thus, irritating foreign substances taken into the mouth; unduly heated, unduly iced, or unduly spiced food and drink; the excessive use or abuse of tobacco and of stimulants; contact of acrid and corrosive acid and alkaline mixtures; {322} the constitutional action of certain medicines, particularly mercury, but likewise bromine, iodine, arsenic, antimony, and, to a slighter extent, other medicinal substances also; inspiration of irritating dust, gases, vapors, steam, and smoke; even hare-lip, cleft palate, and congenital or acquired deformities of the mouth generally,--may all be included in this category.

In the newly-born a special hyperæmia of the mucous membrane has been cited (Billard) as the cause.

Morbid dentition is the most frequent local cause of catarrhal stomatitis in children, but it is an occasional cause in adults likewise. Hence it is frequent from the sixth to the thirtieth month of life; again, between the ages of six and fifteen years, the period of second dentition; and likewise between the eighteenth and twenty-second years, the period for the eruption of the last molars. Deformed, carious, and broken teeth, improper dentistry, wounds and ulcerations of the gums, negligence in cleansing the teeth,--all these contribute their quota as exciting causes. Nurslings occasionally contract the affection from the sore nipples of their nurses. In some instances they acquire it by protracted sucking at an exhausted breast. Protracted crying, from whatever cause, sometimes induces catarrhal stomatitis, not only in nursing children, but in older ones. Prolonged or too frequent use of the voice, whether in talking, reading, singing, or shouting, may be the exciting cause.

Distant irritations of the alimentary tract, exciting catarrhal stomatitis, include stomachic and intestinal derangements of all sorts. Poor food and lack of hygiene on the one hand, and over-feeding, excess of spices, alcohol, and tobacco on the other, are not infrequent exciting causes. Undue excitement, excessive mental emotion, unrestrained passion, deranged menstruation, normal and abnormal pregnancy and lactation, sometimes incite the affection. Slight colds from cold feet or wet clothing give rise to catarrhal stomatitis. It likewise presents as an extension from coryza, sore throat, glossitis, tonsillitis, pharyngitis, and laryngitis.

Deuteropathic or secondary catarrhal stomatitis occurs in various febrile diseases, especially the acute exanthemata--measles, scarlet fever, small-pox; in syphilis, in pulmonary tuberculosis, and in long-continued chronic pneumonia.

Infantile stomatitis is most frequent between the ages of two and twelve months; the stomatitis of adolescents at the periods of dentition; and that of adults when local sources of irritation predominate.

SYMPTOMATOLOGY.--The symptoms in catarrhal stomatitis vary in severity with the intensity and extent of the inflammatory processes.

In the infant the subjective symptoms usually commence with restlessness, fretfulness, and crying. Unwillingness to nurse or inability to do so soon becomes manifest. The child may seize the nipple eagerly with a firm grasp of the lips, but at the first suction lets it drop away with a cry of pain and disappointment. The cause of the pain is made evident on inspection and palpation of the interior of the mouth. The parts are dry, glazed, hot, and tender. So hot is the mouth at times that its heat, conveyed to the nipple in suckling, is sometimes the first intimation of the existence of the malady. Similar conditions often prompt an older {323} child to refuse the teaspoon. This sensitiveness is observed in the tongue and on the inner surface of the cheeks. It increases during movements of the tongue and jaw. Deglutition becomes painful, especially when the food tendered is rather hot or rather cold. There is a grayish-white accumulation of partially detached epithelium on the tongue, sometimes in longitudinal strips, sometimes in a continuous layer. Should the stomatitis be due to dentition, the affected gums will be swollen, hot, and painful. There is usually an augmentation of the secretions in the mouth. Sometimes they flow from the mouth in great quantity, inflaming the lips. These secretions acquire an increased viscidity, so that they become adherent in clammy masses to the tongue, the gums, and the lips. Taste thus becomes impaired, while decomposition of these masses in sitû imparts fetor to the breath; the odor being especially pronounced when the child awakens from a night's sleep, the secretions having accumulated meanwhile more rapidly than they could be discharged. When the secretions of the mouth are not excessive there may be merely a faint mawkish odor to the breath, sweetish in some instances, sour in others. Loss of appetite is usual. Diarrhoea sometimes exists to a moderate degree, attended at times by gaseous distension of the intestines.

In severe cases dependent on morbid dentition swelling of the submaxillary glands and infiltration of the connective tissue may take place. More or less pyrexia becomes evident. In some instances convulsions supervene; either directly from cerebral hyperæmia, or in reflex manner from irritation of the sensitive gingival nerves.

In the adult impairment of taste is one of the earliest subjective symptoms. This symptom is usually accompanied or else closely followed by peculiar viscid and sticky sensations about the tongue, gums, and palate--sensations that excite vermicular motions of the lips and tongue to get rid of the foreign material by expectoration or by deglutition. The taste is usually a bitter one, and the viscid sensations are usually due to accumulations of desquamated epithelium upon the tongue and other structures. An unpleasant odor is sometimes exhaled, the result of decomposition of the excessive secretions.

In the chronic form of the affection, especially as it occurs in the adult, the alterations of taste, the saburral coatings of the tongue, and the fetor of the breath are more marked than in the acute form.

The mucus accumulating during sleep often awakens the patient in efforts at hawking and spitting to detach and expectorate it. These movements are occasionally so violent as to provoke emesis. The disagreeable odor from the mouth is almost continuous.

In uncomplicated cases there is no loss of appetite or impairment of digestion. The presence of these symptoms is presumptively indicative of gastric disease, usually ulcerous or carcinomatous.

The course of the disease varies according to the causes which have given rise to it. When these subside, the stomatitis soon ceases; when they are irremediable, the stomatitis remains incurable. No special period can be mentioned, therefore, for its duration. It terminates, when cured, in complete restoration of the parts to their normal condition. There are no special complications or sequelæ.

PATHOLOGY AND MORBID ANATOMY.--The hyperæmia of the {324} tissues, physiological during the entire process of dentition, is readily provoked into a pathological hyperæmia. Whatever the origin, however, acute catarrhal stomatitis begins, usually, with congestion and tumefaction of the oral mucous membrane. The congestion is sometimes preceded by pallor, as though anæmia from constriction of the capillaries were the initial step in the phenomena. The congestion and swelling are more rarely diffuse than circumscribed; i.e. confined to certain portions of the tissues, especially the gums, which become swollen and painful to contact. The surface is dry and glistening, and the secretion diminished. The mucous membrane is raised in patches here and there where the submucous tissues are the most lax. These patches, irregular in size and configuration, are seen on the tips and edges of the tongue, on the inner surface of the cheeks, at the gingival junctions of the jaws, around the dental margins of the gums, about the angle of the mouth, and on the palate. Sometimes the patches coalesce--to such an extent in rare instances as to cover the entire mucous membrane even of the palate and the gums. Their margins are bright red, their centres yellowish. These elevated patches are due to local accumulation of new-formed cellular elements, perhaps determined by the distribution of capillaries or lymphatics. Intensification of the inflammatory process around or upon them, giving rise to a more abundant cell-proliferation, sometimes occurs; the results presenting macroscopically in ridges or welts of a vivid red, surrounding the patches or traversing them.

The tongue undergoes engorgement, and becomes increased in bulk; exhibiting dentated facets along its edges and around its tip, due to the pressure sustained from the adjoining teeth. Opposite the lines of junction of the two rows of teeth the impression is double. The dividing lines separating the facets project a little, and are opalescent, grayish, or whitish, owing to increased proliferation of epithelium. Similar dentate impressions from a like cause may be seen on the inner surfaces of the cheeks.

The hyperæmia of the parts is soon followed by excessive production of new cellular elements, rendering the now increased secretions turbid; so that the surfaces of the tongue and cheeks become moist again, and covered with a grayish-white, pultaceous form of desquamated epithelium, but slightly adherent, and therefore readily detached by movements of the tongue, lips, and cheeks. In some instances the epithelium becomes raised into minute vesicles, and chiefly on the edges of the tongue, thus presenting a sort of lingual herpes. Excoriations, and even shallow ulcerations, may follow.

Isolated lesions occur. There may be congestion of the palate without tumefaction, its epithelium undergoing detachment in shreds. The congested patches at the dental margins of the gums may become overlaid by opalescent masses of desquamated epithelium, followed by their actual ulceration, and even by detachment of the teeth.

In children the lips may be swollen and excoriated or surrounded by an eruption of herpes. Profuse salivation may occur in a child a few months old when the affection becomes protracted. Febrile movement is rare before the fifth or sixth month.

In chronic stomatitis the tumefaction is usually greater, with distension of the capillaries and hypertrophy of some of the mucous follicles, {325} especially those upon the cheeks and palate. There is also hypertrophy of the lingual papillæ, especially those at the tip of the tongue. Adherent to the gums and the tongue is a yellowish tenacious mucus, composed of squamous epithelia, fat-globules, bacteria, and the usual débris of disorganization. The saliva is secreted in unusual quantities, and sometimes dribbles more or less continuously.

DIAGNOSIS.--Recognition of the conditions described under the head of Pathology and Morbid Anatomy, in the presence of the symptoms described under Symptomatology, renders the diagnosis easy.

Chronic stomatitis may be mistaken for mere indication of gastric catarrh, which is likewise attended with loss of appetite, fetor of breath, and coating of the tongue.

PROGNOSIS.--The prognosis is favorable in almost every instance, recovery being almost universal in the acute form. Stomatitis of dentition subsides with the physiological completion of that process; stomatitis of exanthematic origin ceases with the evolution of the eruptive disorder. In the chronic form ultimate recovery will depend upon the permanency of the existing cause and the extent of the inflammatory new formations.

TREATMENT.--The first indication, as a matter of course, is to obviate the cause, whatever that may be. This, when practicable, usually suffices to bring the malady promptly to a favorable termination.

Intestinal disturbances, whether causative or incidental, must be duly corrected, and the administration of a saline purge is almost always desirable. In addition, resort is made to frequent ablutions with fresh water, warm or tepid, in sprays, gargles, or washes, as may be most convenient or practicable. Emollients (gum-water, barley-water, quinceseed-water), astringents (alum, tannin), and detergents (borax, sodium bicarbonate), may be added, with opiates to relieve pain if need be.

Frequent or continuous suction of fragments of ice usually affords prompt relief to local pain and heat. The anæsthetic properties of salicylic acid have been utilized,[1] one part to two hundred and fifty of water containing sufficient alcohol for its solution.

[Footnote 1: Berthold, cited by Ringer, Handbook of Therapeutics, 10th ed., London, 1883, p. 612.]

Aphthous Stomatitis.

DEFINITION.--Inflammation of the mucous membrane of the interior of the mouth, characterized by small superficial ulcers. These ulcers are irregularly circular or oval, are not depressed below the general surface of the mucous membrane, and support a creamy sebum or exudation. They occupy positions known to be normally supplied with mucous glands.

The classical description of this affection includes the initial eruption of vesicles or groups of vesicles which rupture within a day or two of their appearance, leaving, upon discharge of their contents, the little superficial characteristic ulcers. Modern investigation, however, casts some doubt upon the vesicular character of the initial lesion, and renders it extremely probable that the reiterated expression of this opinion has {326} been a simple deference by writer after writer to the descriptions given by his predecessors. This subject will receive further elucidation more appropriately in describing the pathology and morbid anatomy of the disease.

Aphthous stomatitis may be either idiopathic or symptomatic, discrete or confluent. It is often recurrent, and is sometimes epidemic.

SYNONYMS.--Aphthæ; Vesicular stomatitis; Follicular stomatitis (Billard); Canker sore mouth.

ETIOLOGY.--Aphthous stomatitis occurs at all ages, and is most prevalent during summer heat. In children it is most frequent from the period of the commencement of dentition to the completion of the eruption of the temporary teeth. It is infrequent during the fourth year of life, and is rare after the fifth. It is most apt to appear in pale, delicate, and scrofulous children, especially in such as are predisposed to catarrhal and cutaneous diseases (Billard, Barthez and Rilliet). Sometimes it seems to be hereditary (Barthez). Some individuals are subject to frequent recurrences. Poor food, insufficient clothing, want of due ventilation, lack of cleanliness, and similar deprivations act as predisposing causes. Hence the disease is apt to occur in the crowded wards of hospitals and asylums for children.

Anything that exhausts the physical forces of the adult, such as excessive heat, overwork, anxiety, hardship and privation as in shipwreck, and the drains of menstruation, pregnancy, and lactation, excessive sexual intercourse, etc., may predispose to the disease.

Long-continued debility from severe constitutional maladies, with chronic febrile conditions, such as chronic phthisis, chronic syphilis, chronic enteritis, chronic gastritis, and from diabetes and carcinoma, likewise acts as a predisposing cause, giving rise, during the final stages of the systemic disease, to symptomatic aphthæ, often of the confluent variety. Aphthous stomatitis sometimes accompanies certain of the continued fevers, exanthematous and non-exanthematous.

As exciting causes the following may be cited: gingivitis, from morbid dentition in children, and from neglect of the teeth, dental caries, and dental necrosis in adults; tobacco-smoking; the local contact of acrid substances in food or otherwise; acute gastro-intestinal disorder from improper or tainted food. Excessive humidity of the atmosphere is assigned as a prominent exciting cause of the disease in some countries. This is especially the case in Holland, where it often exists epidemically. The confluent form at these times is said to attack parturient women principally (Ketslaer). Inundations, not only in Holland, but in Hayti, Porto Rico, and in the United States, are sometimes followed by an endemic of aphthous stomatitis. It is believed that the emanations from decayed animal and vegetable matters left ashore on the reflux of the water, produce the morbid conditions which constitute the predisposing cause under such circumstances.

The use of certain drugs--preparations of antimony, for example--sometimes produces a vesicular stomatitis sufficiently analogous to aphthæ to be mentioned in this connection, and only to be distinguished therefrom by the history of the case.

PATHOLOGY AND MORBID ANATOMY.--As has been intimated, the morbid anatomy of aphthæ has long been described as a series of initial {327} vesicles[2] upon the buccal, labial, gingival, or lingual mucous membrane. Their variance from analogous cutaneous vesicles--herpes, for instance--is attributed to anatomical differences in the constitution of the mucous membrane and the skin. The rarity of their detection has been accounted for by the rapid maceration of the epithelium.

[Footnote 2: Tardieu, Hardy and Behier, Barthez and Rilliet, Meigs and Pepper, and many others.]

The general opinion at present, however, is that the apparent vesicle is an inflamed mucous follicle.[3] Some observers contend that it is an inflammation of the mucous membrane pure and simple (Taupin); others consider it an inflammation, sometimes in a follicle, sometimes in the mucous membrane (Grisolle); others, a fibrinous exudation in the uppermost layer of the mucous membrane (Henoch). Some have described it as the analogue of a miliary eruption (Van Swieten, Sauvage, Willan and Bateman); others, of herpes (Gubler, Simonet, Hardy and Behier); others, of ecthyma (Trousseau) and of acne (Worms).

[Footnote 3: Bichat, Callisen and Plenck, Billard, Worms, and others.]

The vesicle of the primary stage, though generally vouched for, is rarely seen by the practitioner, so rapid is the metamorphosis into the aphthous ulcer. Its very existence is positively denied by several authorities (Vogel, Henoch), and Vogel states that he has never, even upon the most careful examination, discovered a real vesicle upon the mucous membrane of the mouth--one which, upon puncture, discharged thin fluid contents and then collapsed.

Beginning in a few instances, only, in a simple stomatitis, the initial anatomical lesion presents as a red, hemispherical elevation of epithelium one to two millimeters in diameter, and barely perceptible to the touch of the finger, though described by the patient as positively appreciable to the touch of the tongue. Believed to have been transparent or semi-transparent at first, its summit is usually opaque when first seen by the medical attendant, appearing as a little white papule. Billard describes a central dark spot or depression--the orifice of the duct of the inflamed follicle, as he considers it. Worms and others, however, who likewise attribute the little tumor to an inflamed follicle, have failed to recognize any such central depression. There may be but four or five of these papules; rarely are there more than twenty. Diffuse inflammation between them is rare. A few new papules are seen on the second day, perhaps a few fresh ones on the third day. Eventually, contiguous desquamations coalesce into an irregular excoriated or ulcerated surface. These appearances and processes may be summed up as hyperæmia, increased cell-proliferation into circumscribed portions of the mucous structures, with distension of the epithelium (dropsical degeneration?), rupture, and ulceration.

This is the stage at which the local lesion usually comes under professional notice as a superficial circular or ovoidal ulceration or patch, with irregularly rounded edges and an undermined border of shreddy epithelium. It is level with the surface or but slightly tumefied, and is usually surrounded by an inflammatory areola that gives it a slightly excavated aspect. Sometimes this is a narrow red rim, and sometimes it is a delicate radiating arborescence of several millimeters. Adjacent ulcerations coalesce and produce irregularly elongated losses of substance. The floor {328} of the ulcer is covered with an adherent semi-opaque or opaque lardaceous mass, sometimes grayish-white, sometimes creamy or yellowish-white when unadmixed with other matters; the color depending more or less upon the number of oil-globules present, the result of fatty degeneration of the epithelium.

For a few days, three to five or more, the surface of the ulcer increases slightly by detachment of its ragged edges, eventually leaving a clean-cut sore, gradually reddening in color, with an inflammatory margin indicative of the reparative process. Repair steadily progresses by the reproduction of healthy epithelium from periphery to centre, so that within a day or two the size of the ulcer becomes diminished to that of a pinhead; and this is promptly covered over, leaving a red spot to mark its site, until, in a few days more, the color fades in its turn, and no trace of the lesion remains. The period of ulceration is prolonged to one or more weeks in some subjects, chiefly those of depraved constitution.

It was the uniform configuration of the initial lesions, their invariable seat, and the central depression which he detected, that led Billard to the opinion that the so-called eruption or vesicle was an inflamed mucous follicle. This view was further supported by the fact that the disease does not occur in the new-born subject, in whom the lymphatic glands and follicles of the digestive tract are barely developed, while it does occur after the fifth or sixth month of life, up to which time these structures are growing rapidly, and thus predisposing the infant to this peculiar disease by reason of the physiological nutritive hyperæmia.

Discrete aphthæ are found principally in the sides of the frenum and on the tip and sides of the tongue; on the internal face of the lips, the lower lip particularly, near their junction with the gums; on the internal face of the cheeks, far back, near the ramus of the jaw; upon the sides of the gums, externally and internally; on the summit of the gums of edentulous children (Billard); exceptionally upon the soft palate; in rare instances upon the pharynx.

Confluent aphthæ appear in the same localities as are mentioned above, and are much more frequent in the pharynx and oesophagus than are discrete aphthæ. They are said to be found occasionally in the stomach and in the intestinal canal.

In the confluent form of the disease the aphthæ are much more numerous, and the individual ulcerations run into each other; coalescing into elongated ulcers, especially upon the lower lip and at the tip of the tongue.

SYMPTOMATOLOGY, COURSE, DURATION, TERMINATIONS, COMPLICATIONS, AND SEQUELÆ.--The discrete form of the affection is rarely attended by constitutional disturbance of any gravity, and such disturbance, slight as it may be, is much more frequent in children than in adults. The local manifestation gradually wanes from periphery to centre in from eight to ten days, the patches changing in color from grayish to yellow, becoming translucent, and losing their red areola, until nothing but dark-red spots remain to mark their site. These spots fade in time, removing all trace of lesion.

Aphthous stomatitis of secondary origin attends conditions of serious constitutional disturbance--circumstances under which it is incidental and not causal.

The confluent form, unless exceedingly mild, is attended by symptoms {329} of gastric or intestinal derangement--viz. coated tongue, thirst, salivation, acid or acrid eructations, nausea, perhaps vomiting, indigestion, and constipation or diarrhoea, as may be. The vomiting in these instances is usually attributed to the presence of aphthæ in the oesophagus and stomach, and the diarrhoea to their presence in the intestines.

Severer cases present, in addition, febrile phenomena, restlessness, loss of appetite, and unhealthy fecal discharges.

The constitutional symptoms precede the local manifestations in some instances by a number of days.

Confluent epidemic aphthous stomatitis, as it occurs in parturient women, is described (Guersant) as commencing with rigors, headache, and fever. The local symptoms are very severe. Pustules form upon the palate and pharynx. Deglutition becomes painful and difficult. Vomiting and painful diarrhoea occur, indicating extension of the disease to the stomach and the intestines. Typhoid conditions may supervene, and continue as long as three weeks, even terminating fatally.

The earliest local symptoms consist in some degree of discomfort and heat, to which severe smarting becomes added at the period of ulceration. The little sores, no matter how minute they may be, are exceedingly painful to the touch, even to the contact of the tongue. Mastication thus becomes painful, and even impracticable, in the adult; and suction at the breast or the bottle difficult and painful in the infant. The mouth of the infant is so hot that its heat is imparted to the nipple of the nurse, whose sensations in nursing sometimes furnish the earliest indication of the disease. Indeed, the heat of the child's mouth at this time, and the acridity of the buccal secretions, are often sufficient to irritate and inflame the nipple, and even to produce superficial excoriation. The general mucous secretions of the mouth are usually augmented. Hypersalivation is much less frequent.

The course of the disease is mild as a rule. The chief inconvenience is the difficulty in alimentation consequent on the pain in mastication and in swallowing.

The duration of the affection in idiopathic cases varies, as the rule, from four to seven days, counting from the first appearance of the local lesion to the complete repair of the succeeding ulceration. Individual cases are often more protracted. Successive crops of aphthæ may prolong the disease for many days. In confluent aphthæ the course is slower and the disease less amenable to treatment; ulceration often continuing longer than a week, and recovery requiring twelve or fifteen days. The duration in consecutive cases varies with the nature of the underlying malady. In individuals seriously debilitated by protracted constitutional disease, as in the subjects of phthisis, the affection may continue, with intermissions and exacerbations, as long as the patient lives. The termination of the individual ulcerations is in repair.

There are no special complications. The accompanying stomatitis is usually a gingivitis simply, and is apt to be circumscribed when more extensive.

There are no sequelæ. Sometimes labial herpes or similar ulcerations follow, which are likewise sore and painful.

DIAGNOSIS.--The isolated patches of the discrete form are usually sufficiently characteristic to establish the diagnosis.

{330} In children the gums are usually seen to be congested, swollen, moist, and glistening. Sometimes they are even sanious. This condition is deemed of great importance in cases of small, solitary aphthæ concealed in the sinus between gums and lips (Rilliet).

Confluent aphthæ may be mistaken for ulcerative or ulcero-membranous stomatitis, especially when the emanations from a coated tongue exhale a disagreeable or fetorous odor.

From thrush--with which it is most frequently confounded--it is to be discriminated by the absence, upon naked-eye inspection, of the peculiar curdy-like exudations to be described under the appropriate section, and under microscopic inspection by the lack of the peculiar thrush-fungus (Oïdium albicans).

PROGNOSIS.--Recovery is usually prompt in discrete cases, but relapses are not infrequent. In confluent cases recovery is dependent upon the character of the constitutional disorder by which the local disease has been caused or with which it is associated, and is therefore much slower.

The disease is grave in certain epidemic confluent forms, such as are described as occurring in Holland and elsewhere under conditions alluded to. Parturient women under such circumstances occasionally succumb to the typhoid condition into which they are thrown. When following measles there is some danger of laryngitis, and the case becomes grave. Oedema of the larynx is sometimes produced.

TREATMENT.--Very simple treatment suffices in the discrete form of the disease. A mild antacid, or even an emetic, may be indicated when there is gastric derangement or disturbance; or a mild laxative when the patient is costive. Castor oil, rhubarb, or magnesia may be given, followed, if need be, by an astringent if diarrhoea should occur. A little opium may be administered if requisite. The diet should be quite simple and unirritating. Cold milk is often the very best diet, especially while the mouth remains quite sore.

Topical treatment in the milder cases may be limited to simple ablutions, by rinsing or by spray, with water, cold or tepid as may be most agreeable to the patient. A little opium may be added when the parts are painful or tender. In severer cases an antiseptic wash may be substituted, as the sodium sulphite or hyposulphite, thirty grains to the ounce, creasote-water, or the like.

Demulcent washes of elm, sassafras-pith, or flaxseed are often more soothing than simple water. Pellets of ice from time to time are quite refreshing and agreeable. Occasional topical use of borax or alum, applied several times a day by means of a hair pencil, soft cotton wad, or the like, is often useful, care being taken to touch the sores lightly, and not to rub them. If the course toward repair is retarded, the parts may be touched lightly with silver nitrate in stick or in strong solution (60 grains), or washed more freely, two or three times a day, with a weaker solution, five or ten grains to the ounce of distilled water. Cupric sulphate, ten grains to the ounce, zinc sulphate, twenty grains to the ounce, mercuric chloride, one grain to the ounce, or potassium chlorate, twenty grains to the ounce, may be used as local applications, repeated at intervals of four or five hours. Iodoform has been highly recommended of late.

{331} The confluent variety requires constitutional treatment adapted to the underlying malady. Nutritious diet is often demanded, together with tonics, such as iron and quinia, or even stimulants, wine or brandy. Topically, cauterization with silver nitrate is more apt to be indicated, and to be indicated more promptly than in the discrete form. Potassium chlorate in doses of one or more grains may often be given with advantage, at intervals of from four to two hours.

Stomatitis Parasitica.

DEFINITION.--An exudative inflammation of the interior of the mouth, due to the development upon the mucous membrane of a parasitic vegetable confervoid growth, the Oïdium albicans (Robin).

SYNONYMS.--Stomatitis cremosa; Stomatitis pseudo-membranosa; Thrush; Muguet of the French; Schwämmchen of the Germans.

HISTORY.--Thrush was long regarded as a pseudo-membranous variety of stomatitis, and was likewise confounded with other varieties of stomatitis, especially aphthæ, its differentiation from which will be rendered apparent by a study of its etiology and morbid anatomy.

The microscopic researches of Berg[4] of Stockholm upon the minute structure of the supposed pseudo-membrane developed the fact that it was largely composed of certain cryptogams. This growth was named Oïdium albicans by Prof. Ch. Robin,[5] by whom it had been subjected to minute study.

[Footnote 4: Ueber die Schwämmchen bei Kindern, 1842--Van der Busch's translation from the Swedish, Bremen, 1848.]

[Footnote 5: Histoire naturelle des Végétaux parasites, Paris, 1853.]

Later observers consider the oïdia in general simply transitional forms in the life-history of fungi otherwise classified. According to Grawitz, the O. albicans is a stage of the Mycoderma vini, his experiments having shown that on cultivation the filaments germinate like Torula and Mycoderma, and that the latter can be grown in the epithelium of the mucous membrane.[6]

[Footnote 6: Ziegler, A Text-book of Pathological Anatomy and Pathogenesis, translated by Macalister, vol. i. p. 319, London, 1883.]

ETIOLOGY.--Thrush is usually a symptomatic disease, secondary to an {332} acid condition of the fluids of the mouth. Athrepsia (Parrot, Meigs and Pepper), or innutrition, is the presumable predisposing cause. Negligence in maintaining cleanliness of the mouth and of the articles which are placed in it is regarded as the main exciting cause. It occurs both in the adult and in the infant, but it is much more frequent in infancy and in early childhood. It is most frequently encountered in asylums and hospitals for children, being often transmitted from child to child by the nurse or by means of the feeding-bottle. The poor health of the child seeming less accountable for the disease than the unsanitary condition of the wards, buildings, and surroundings, it is consequently much less frequent in private than in public practice. It is more frequent in the first two weeks of life than later. Seux observed it within the first eight days in 394 cases out of 402 (Simon). It is much more frequent during summer than at any other season, more than half the cases (Valleix) occurring at that portion of the year.

In senile subjects, in adults, and in children more than two years of age it is cachectic, and observed chiefly toward the close of some fatal and exhausting disease, such as diabetes, carcinoma, tuberculosis, chronic pneumonia, enteric fever, puerperal fever, erysipelas, chronic entero-colitis and recto-colitis, and pseudo-membranous sore throat. It is sometimes observed in the early stage of enteric fever.

Meigs and Pepper, apparently following Parrot, deem the central cause to lie in a certain failure of nutrition under which the general vitality slowly ebbs away. They are inclined[7] to recognize a causal factor in a deficiency in the supply of water in much of the artificial food administered to young subjects. The normal acidity of the fluids of the mouth of the newly-born (Guillot, Seux) is not sufficiently counteracted until saliva becomes abundant. Premature weaning, entailing, as it often does, the use of improper foods, renders the child liable to gastro-intestinal disorders. To this add want of care of the bottle and nipples, of the teaspoon or pap-boat, and of the mouth itself, and the conditions are fulfilled in fermentations of remnants of milk taking place without and within, which produce the acid condition of the fluids and secretions of the mouth said always to accompany and precede the development of the disease (Gubler).

[Footnote 7: A Practical Treatise on the Diseases of Children, 7th ed., Philada., 1882.]

The theory of contagiousness seems established (Guillot, Berg, Gubler, Robin, Trousseau). This has been further demonstrated by experiments upon sheep (Delafeud), in which thrush has been implanted whenever the animals were unhealthy, but not otherwise.

PATHOLOGY AND MORBID ANATOMY.--The mucous membrane of the mouth within a few hours after its invasion by thrush is seen to be covered to some extent by minute masses of a granular curdy substance adherent to the tissues, which often bleed slightly when the substance is forcibly removed.

In children much reduced by inanition or severe disease, much of the deposit soon coalesces into a membraniform product, grayish or yellowish from rarefaction by the air, or even brownish from admixture of blood. By the same time the general congestion of the mucous membrane will have subsided into the pallor of anæmia. Though tolerably adherent when fresh, the deposit when older often becomes loosened {333} spontaneously, so that it may be removed by the finger in large flakes without producing any hemorrhage whatever.

The characteristic masses present both as delicate roundish flakes, isolated, not larger than a pinhead, and as confluent patches several times as large and more irregular in outline. These masses under microscopic inspection are seen to be composed of the filaments and spores of a confervoid parasitic plant, the Oïdium albicans, enclosing altered epithelia in various conditions. This parasitic growth does not become developed upon healthy mucous membrane with normal secretory products. Acidity of the fluids and exuberance of epithelium are the requisites for its production, whatever be the cause. The acidity of the fluids irritates the mucous membrane upon which they lie. This irritation induces abnormal proliferation of epithelium, upon which the spores of the cryptogam then germinate. Dissociated epithelial cells become proliferated at the surface of the mucous membrane, between which and upon which both free and agglutinated spores accumulate. From these spores sprout out simple and ramified filaments in compartments containing moving granular elements. (For the minute detailed anatomy of these filaments and spores the reader is best referred to Robin's work on Vegetable Parasites.)

It may suffice here to mention that the filaments are sharply-defined tubercles, slightly amber-tinted, of a mean diameter of between four and three millimeters, simple while immature and branched when fully developed. These tubules are filled with link-like groups of elongated cells in compartments, giving them an appearance of regular constriction at the junctions of adjoining groups of cells. Surrounding these tubules are groups of spheroid or slightly ovoid spores from five to four millimeters in diameter. Each spore contains one or two granules and a quantity of fine dust. This cryptogamic growth is developed in the proliferated cells of epithelium. The filaments in their further growth separate the epithelia, and even penetrate them. Thence they penetrate the mucous membrane and the submucosa (Parrot).

The mucous membrane beneath the growth is red, smooth, and glistening. Papillæ are sometimes prominent. It is not excoriated unless the growth has been removed with some violence, when, as noted, it may bleed slightly. Duguet and Damaschino have recently encountered cases associated with a special ulceration of one of the palatine folds; the former in enteric fever, the latter in a primitive case. The growth is quickly reproduced after removal--even within a few minutes when the secretions are very acid.

The glossal mucous membrane is usually the tissue first involved, the specks being more numerous at the tip and edges of the tongue than at its central portion. The glands at the base of the tongue may become invaded. From the tongue extension takes place to the lips, the cheeks, the gums, and the palate, hard and soft. The growth is especially prolific in the folds between lips and gums and between cheeks and gums. Sometimes the parts mentioned become involved successively without actual extension. In several recently reported instances occurring during enteric fever,[8] the affection began on the soft palate, tonsils, and pharynx, and then progressed anteriorly toward the tongue, the cheeks, and the lips.

[Footnote 8: Duguet, Soc. Méd. des. Hôp., Mai 11, 1883; Rev. mens., Juin 1, 1883, p. 187.]

{334} But there is no limitation of the disease to these structures. The growth may cover the entire mucous membrane of the mouth. From the mouth it may reach the lateral walls of the pharynx, and in rare instances the posterior wall of the pharynx. The product is said to be more adherent on the pharynx (Reubold) than in the mouth. From the pharynx it may reach the epiglottis, and even the larynx (Lelut), in which organ it has been seen upon the vocal bands (Parrot). It has never been observed in the posterior nares or at the pharyngeal orifice of the Eustachian tube. It flourishes best, therefore, upon squamous epithelium. In infants much reduced, Parrot has seen ulceration in the neighborhood of the pterygoid apophyses, but attributable to the cachectic state of the child, and not to the disease in the mouth.

In many cases--in as large a proportion as two-thirds, according to some observers--the oesophagus becomes invaded, either in irregular longitudinal strips or in rings, in all instances (Simon) terminating a little above the cardia. In exceptional cases the entire mucous surface of the oesophagus may be covered with the product (Seux). It has been seen in the stomach (Lelut, Valleix), and is even said to be developed there (Parrot), presenting as little yellow projections, isolated or contiguous, from the size of millet-seeds to that of peas, and usually located along the curvatures, especially the smaller curvature and cardia (Simon).

In instances still more rare it is found in the intestinal canal (Seux), even at the anus (Bouchut, Robin), and thence upon the genitalia. In a child thirteen days old, Parrot found it in the pulmonary parenchyma at the summit of the right lung, where it had probably been drawn by efforts of inspiration.

The nipple of the nurse often becomes covered with the growth (Gubler, Robin, Trousseau, Simon).

SYMPTOMATOLOGY.--In infants the earliest symptom is distress during nursing, the nipple being seized repeatedly, and as frequently released with cries of pain and disappointment. This cry is hoarse when the vocal bands are involved.

The constitutional symptoms depend upon the underlying malady, and may of course vary with its character. Thus we may have the symptoms of simple diarrhoea, gastro-enteritis, or entero-colitis on the one hand, and of tuberculosis and other diseases elsewhere enumerated on the other. Cachectic children, especially in asylum and hospital practice, lose flesh, and their skins become harsh, dry, and inelastic from loss of fluids (Meigs and Pepper). The genitalia, the anus, and the adjacent parts become eroded by the acridity of the discharges, and then become covered with the growth.

The disease rarely lasts longer than eight days in strong children that can be well cared for. It may continue indefinitely, on the other hand, in cachectic children; that is to say, for several months or until the patient succumbs, as may be. Death occurs usually from the causal disease, and not as a result of the morbid condition of the mouth.

DIAGNOSIS.--In the Infant.--Examination of its mouth to detect the cause of the child's inability to nurse reveals congestion of the mucous membrane, intense and often livid in severe cases. It is first noticed at the extremity of the tongue. When the congestion is general it is darkest in the tongue. This livid congestion may extend over the entire {335} visible mucous membrane, save upon the hard palate, where it is tightly adherent to the periosteum, and upon the gums, where it is rendered tense by the approach of erupting teeth. The papillæ at the tip and sides of the tongue are very prominent. Sometimes the organ is quite dry, even sanious, while it is painful to the touch. The reaction of the secretions of the mouth is acid instead of alkaline, and the parts are hot and very sensitive.

Two or three days later the circular milky-white or curdy spots or slightly prominent and irregularly-shaped flakes or patches may be seen on the upper surface of the tongue toward the tip and inside the lips and the cheeks, especially in the grooves connecting gums and lips and gums and cheeks. The surrounding mucous membrane is unaltered in mild cases, and there is no evidence of other local disorder or of any constitutional involvement. In severe cases the entire mucous membrane is dry and deeply congested.

The affection can be positively discriminated from all others by microscopic examination of the deposit, which reveals the presence of the cryptogam described.

TREATMENT.--In infants, artificial nourishment, whether with milk of the lower animals or prepared food of whatever composition, should be given up, if possible, and a wet-nurse be supplied. If this procedure be impracticable, the least objectionable mode of preparation of cow's milk should be employed (and this will vary with the practice of the physician), and the utmost circumspection should be maintained in securing the cleanliness of the vessels in which it is prepared, the bottle from which it is given, and the nipple which is placed in the child's mouth. Should the sugar and casein in the milk appear to keep up the disease, weak soups may be substituted for the milk diet until it has subsided. Weiderhofer advises artificial nourishment, by way of a funnel inserted in the nasal passages, in case the child should refuse to swallow. Deglutition is excited in a reflex manner when the milk or other fluid reaches the pharynx.[9]

[Footnote 9: Journ. de Méd. Bordeaux, Juin 10, 1883.]

The local treatment should consist in careful removal of the patches from time to time--say every two or three hours--with a moistened soft rag. This must be done without roughness of manipulation. In addition to this, the parts may be washed or painted every hour or so with an alkaline solution for the purpose of neutralizing the acidity of the fluids of the mouth. For this purpose borax is most generally used, in the proportion of twenty grains to the ounce of water or the half ounce of glycerin. Sodium bicarbonate or sodium salicylate may be substituted for the sodium borate. The use of honey in connection with the drug is calculated to promote acidity by fermentation of its glucose, and is therefore, theoretically, contraindicated.

Adults may use washes, gargles, or sprays of solutions of sodium borate or of sodium bicarbonate.

The constitutional treatment in each case must be adapted to the nature of the underlying malady which has favored the local disease, with resort in addition to the use of quinia, iron, wine, spirit, and beef-essence. The hygienic surroundings should be made as sanitary as possible.

{336} Stomatitis Ulcerosa.

DEFINITION.--Inflammation of the interior of the mouth, usually unilateral, eventuating in multiple ulcerations of the mucous membrane.

SYNONYMS.--Fetid stomatitis, Phlegmonous stomatitis, Putrid sore mouth, Stomacace, are synonymous terms for idiopathic ulcerous stomatitis. Ulcero-membranous stomatitis, Mercurial stomatitis (Vogel), are synonymous terms for the deuteropathic variety of the disease.

ETIOLOGY.--The principal predisposing cause of the disease is to be found in ochlesis; the contaminating atmosphere of crowded dwellings and apartments insufficiently ventilated; uncleanliness; insufficiency of proper clothing; unhealthy food, and the like. It prevails epidemically in crowded tenements, schools, prisons, asylums, and hospitals; in garrisons and in camps; in transports and men-of-war. It is often propagated by contagion, but whether by infection or actual inoculation seems undetermined. Measles is an active predisposing cause. Feeble individuals are the most liable to the disease. It occurs at all ages. In civil life it is most frequent between the ages of four and ten years. Sometimes more girls are affected than boys (Meigs), and sometimes it is the more prevalent among boys (Squarrey). Autumn is the season of greatest prevalence.

Carious teeth, fracture and necrosis of the jaw (Meigs), and protracted catarrhal stomatitis are among the chief exciting causes. Irregular dentition is sometimes the exciting cause; and this may occur at the first and second dentition or at the period of eruption of the last molars.

PATHOLOGY.--The anatomical lesion is the destructive inflammation of portions of the mucous membrane of the mouth, leaving ulceration on detachment of the eschars. It usually commences as a gingivitis. At two periods of life--namely, from the fourth to the eighth year of life, and from the eighteenth to the twenty-fifth year--it is apt to be ulcero-membranous, a condition asserted to be altogether exceptional at other periods (Chauffard).

A diffuse fibro-purulent infiltration of the lymph-spaces of the mucosa is regarded as the first step in the pathological process. This infiltration is sufficiently abundant to compress the capillary vessels of the tissues, and thus arrest the circulation (Cornil et Ranvier). All those localized portions of mucous membrane from which the circulation is cut off perish and are discharged in fragments. The ulcers thus left are grayish, granular, and sanious, with thin, irregularly dentated borders a little undermined, through which pus can be expressed on pressure. The usual cryptogams of the oral cavity, in various stages of development, are in great abundance in the grayish detritus, which likewise contains altered red and white blood-corpuscles.

According to some observers (Caffort, Bergeron), the first evidence of the disease is an intensely congested erythematous patch, upon which one or more pustules present, point, and rupture promptly, leaving the characteristic ulcerations.

For some indeterminate reason, the ulcerations are mostly unilateral, and occur much the more frequently on the left side. The principal {337} primal points of ulceration are upon the external borders of the gums, more frequently those of the lower jaw, and upon the corresponding surface of the cheek and lip--the cheek much oftener than the lip. Thence ulceration may extend to the tongue, less frequently to the palate. The ulcerative process follows the outline of the gums, baring the bases of the teeth to a variable extent, so that they seem elongated. On the cheek the patch of inflammation is generally oval, the longest diameter being antero-posterior, and the most frequent position is opposite to the last molar.

Each ulcer is surrounded by an intensely red areola, beyond which the tissues are succulent and tumid from collateral inflammatory oedema, often giving the ulcers an appearance of great depth; but when the detritus is discharged they are seen to have been superficial. Detachment of the necrosed segments of mucous membrane takes place by gradual exfoliation from periphery to centre. Sometimes detachment occurs in mass, usually in consequence of friction or suction. The ulcers, gingival and buccal, bleed easily when disturbed. They may remain separate, or may coalesce by confluence of interposing ulcerations extending across the furrow between gum and cheek or lip. The adjoining side of the tongue sometimes undergoes similar ulceration from behind forward, inoculated, most likely, by contact with adjoining ulceration. In rare instances, neglected cases most probably, the ulceration may extend to the palatine folds, the tonsils, and the soft palate.

SYMPTOMATOLOGY.--The affection usually begins without any constitutional symptoms. Young infants sometimes present slight febrile symptoms, with impairment of appetite and general languor. Fetid breath, salivation, and difficulty in deglutition are usually the first manifestations of the disease to attract attention. The mouth will be found to be hot, painful, and sensitive to the contact of food. Infants often refuse food altogether, though usually they can be coaxed to take liquid aliment. Larger children and adults complain of scalding sensations. They find mastication painful, and cannot chew at all on the affected side. The salivation is excessive, the saliva bloody and often extremely fetid. When swallowed, this fetid saliva causes diarrhoea. The cheeks sometimes become swollen, and the submaxillary connective tissue oedematous. Adenitis takes place in the submaxillary, retro-maxillary, and sublingual glands of the affected side. Sometimes the other side becomes affected likewise, but to a less extent. The glands do not suppurate, but the adenitis may remain as a chronic manifestation in scrofulous subjects.

The disease, left to itself, will often continue for a number of weeks, or even months as may be, unmodified even by intercurrent maladies (Bergeron). Long continuance may result in partial or complete disruption of the teeth, or in local gangrene, or even in necrosis of the alveoli (Damaschino). Properly managed, the ulcers become cleansed of their detritus, and within a few days heal by granulation, their position long remaining marked by delicate red cicatrices upon a hard and thickened substratum. Repeated recurrences are sometimes observed.

DIAGNOSIS.--The appearances of the gums and adjoining structures described under the head of Pathology establish the diagnosis. The usually unilateral manifestation and the peculiar fetid odor distinguish it from severe forms of catarrhal stomatitis. From cancrum oris it is {338} distinguished by the absence of induration of the skin of the cheek over the swollen membrane, and by the succulence and diffuseness of the tumefaction. From mercurial stomatitis it is discriminated by the history, and by the absence of the peculiar manifestations to be discussed under the head of that disease.

PROGNOSIS.--The prognosis is good, the disease being susceptible of cure in from eight to ten days in ordinary cases. When due nutrition is prevented by the pain in mastication and deglutition, and in much-reduced subjects, the disease may continue for several weeks. It is in these cases that detachment of the teeth takes place, with periostitis and necrosis of the alveoli. Protracted suppuration and failure in nutrition may lead to a fatal result, but such a termination is uncommon.

TREATMENT.--Fresh air, unirritating and easily digestible food, the best hygienic surroundings practicable, attention to secretions from skin and bowels by moderate and judicious use of ablutions, diaphoretics, and laxatives, with the internal administration of cinchona or its derivatives, with iron and cod-liver oil, comprise the indications for constitutional treatment.

Locally, demulcent mouth-washes are called for, containing astringents, detergents, or antiseptics. Acidulated washes are more agreeable in some instances. For antiseptic purposes, however, sprays and douches may be used of solutions of potassium permanganate, boric acid, carbolic acid, or salicylic acid. Gargles of potassium chlorate, ten or twenty grains to the ounce, are highly recommended, as well as the internal administration of the same salt in doses of from two to five grains three times a day for children, and of ten to twenty grains for adults.

If the sores are slow to heal, the ulcerated surfaces may be touched once or twice daily with some astringent, such as solution of silver nitrate (ten grains to the ounce), or, if that be objectionable, with alum, tincture of iodine, or iodoform.

Prompt extraction of loose teeth and of loose fragments of necrosed bone is requisite.

Stomatitis Gangrenosa.

DEFINITION.--A non-contagious, deuteropathic inflammation of the interior of the mouth, almost invariably unilateral, and characterized by a peculiar gangrenous destruction of all the tissues of the cheek from within outward.

SYNONYMS.--Gangrenous stomatitis; Gangrena oris; Grangrenopsis; Cancrum oris; Stomato-necrosis; Necrosis infantilis; Gangrene of the mouth; Gangrenous erosion of the cheek; Noma; Buccal anthrax; Aquatic cancer; Water cancer; Scorbutic cancer; Sloughing phagedæna of the mouth.

HISTORY.--The most important work upon the subject was published in 1828, from the pen of Dr. A. L. Richter,[10] whose accurate historical account of the disease was in great part reproduced, with additions thereto, by Barthez and Rilliet in their Treatise on the Diseases of Infants, Paris, 1843, and quoted by nearly all subsequent writers on the {339} theme. From these records it appears that the first accurate description of the affection was given in 1620 by Dr. Battus, a Dutch physician, in his Manual of Surgery. The term aquatic cancer, water-kanker, bestowed on it by van de Voorde, has been generally followed by the physicians of Holland, although van Swieten (1699) properly designated it as gangrene. J. van Lil termed it noma, as well as stomacace and water-kanker, and cited a number of Dutch physicians who had observed its epidemic prevalence. The majority of more recent observers, however, deny its epidemic character.

[Footnote 10: Der Wasserkrebs der Kinder, Berlin, 1828; further, Beiträg zur Lehre vom Wasserkrebs, Berlin, 1832; Bemerkungen über den Brand der Kinder, Berlin, 1834.]

Of Swedish writers, Lund described it as gangrene of the mouth; Leutin, under the name of ulocace. In England, Boot was the first to write of gangrene of the mouth, and was followed by Underwood, Symmonds, Pearson, S. Cooper, West, and others.

In France it has received great attention. Berthe[11] described it as gangrenous scorbutis of the gums; Sauvages (1816) as necrosis infantilis. Baron in 1816 published[12] a short but excellent account of a gangrenous affection of the mouth peculiar to children; and Isnard presented in 1818 his inaugural thesis on a gangrenous affection peculiar to children, in which he described, simultaneously, gangrene of the mouth and gangrene of the vulva. Then followed Rey, Destrees (1821), Billard (1833), Murdoch, Taupin (1839), and others, until we reach the admirable description by Barthez et Rilliet, from which the present historical record has been chiefly abstracted.

[Footnote 11: Mémoires de l'Académie royale de Chirurgie, Paris, 1774, t. v. p. 381.]

[Footnote 12: Bullétins de la Faculté de Médecine de Paris, 1816, t. v. p. 161.]

German physicians likewise have largely studied the subject. De Hilden, A. G. Richter, C. F. Fischer, Seibert, and many others preceded A. L. Richter, whose important contribution to the literature and description of the disease has been so highly extolled by Barthez and Rilliet.

In America the disease has been best described by Coates, Gerhard, and Meigs and Pepper, all of Philadelphia.

(For extensive bibliographies the following sources should be consulted in addition to those cited: J. Tourdes, Du Noma ou du Sphacèle de la Bouche chez les Enfants, Thèse, Strasbourg, 1848: A. Le Dentu, Nouveau Dictionnaire de Médecine et de Chirurgie pratique, article "Face," Paris, 1871.)

ETIOLOGY.--Almost exclusively a disease of childhood, gangrenous stomatitis is exceedingly rare in private practice, and very infrequent at the present day even in hospital and dispensary practice. Lack of hygienic essentials of various kinds, impoverishment, long illnesses, and debilitating maladies in general are the predisposing causes. It is sometimes endemic in hospitals and public institutions, but rarely, if at all, epidemic. It is not generally deemed contagious, though so considered by some writers. It appears to have been more frequent in Holland than elsewhere, to be more frequent in Europe generally than in the United States, and now much less frequent in the United States than formerly. To recognition of the predisposing causes and to their abolition and avoidance may probably be attributed its diminished frequency all over the world. Though attacking children only as a rule, it has been observed in adults (Barthez et Rilliet, Tourdes, Vogel). Nurslings are not liable to the disease. Though occurring occasionally earlier in life, the greatest period of prevalence is {340} from the third to the fifth or sixth year of age, and thence, with diminishing frequency, to the twelfth and thirteenth years. It is probably equally frequent in the two sexes, though the majority of authors have described it as more frequent in females.

Healthy children are not attacked. Even in delicate children it is so rarely idiopathic that this character is utterly denied it by many observers. The disease which it follows, or with which it becomes associated, may be acute or chronic. According to most writers, it occurs with greatest frequency after measles. It follows scarlatina and variola much less often. It is observed likewise after whooping cough, typhus fever, malarial fever, entero-colitis, pneumonitis, and tuberculosis. Excessive administration of mercury has been recognized as an exciting cause, some cases of mercurial stomatitis progressing to gangrene.

According to Barthez et Rilliet, acute pulmonary diseases, and especially pneumonia, are the most frequent concomitant affections, and are usually consecutive.

SYMPTOMATOLOGY, COURSE, DURATION, TERMINATIONS, COMPLICATIONS, AND SEQUELÆ.--The disease usually becoming manifested during other disease, acute or chronic, or during convalescence therefrom, there are no special constitutional symptoms indicating its onset. Hence considerable progress may be made before its detection. The earliest local characteristic symptom distinguishing gangrenous stomatitis is a tense tumefaction of one cheek, usually in proximity to the mouth. The lower lip is generally involved, thus rendering it a matter of difficulty to open the mouth. This tumefaction in some instances progresses over the entire side of the face up to the nose, the lower eyelid, and even out to the ear in one direction, and down to the chin, and even to the neck, in the other. Before the parts become swollen externally, ulceration will have taken place to some extent in the mucous membrane, but usually without having attracted special attention, the subjective symptoms having been slight. A gangrenous odor from the mouth, however, is almost always constant. Its presence, therefore, should lead to careful investigation as to its seat and cause. The gums opposite the internal ulcer become similarly affected in most instances, and undergo destruction, so that the teeth may become denuded and loosened, and even detached, exposing their alveoli. The bodies of the maxillary bones suffer in addition in some instances, and undergo partial necrosis and exfoliation.

It is maintained (Löschner, Henoch) that in some instances there is no involvement of the mucous membrane until the ulcerative process has reached it from the exterior.

The tumefied portions of the check and lip are pale, hard, unctuous, and glistening. They are rarely very painful, and often painless. On palpation a hard and rounded nodule one or two centimeters in diameter can be detected deep in the central portion of the swollen cheek.

From the third to the sixth day a small, black, dry eschar, circular or oval, becomes formed at the most prominent and most livid portion of the swelling, whether cheek or lip. This gradually extends in circumference for a few days or for a fortnight, sometimes taking in almost the entire side of the face or even extending down to the neck. As it enlarges the tissues around become circumscribed with a zone intensely red. The internal eschar extends equally with the external one. Eventually, the {341} eschar separates, in part or in whole, and becomes detached, leaving a hole in the cheek through which are seen the loosened teeth and their denuded and blackened sockets.

During this time the patient's strength remains tolerably well maintained, as a rule, until the gangrene has become well advanced. Intelligence usually remains good. Many children sit up in bed and manifest interest in their surroundings. Others lie indifferent to efforts made for their amusement. Some exhibit insomnia and delirium. The pulse is small and moderately frequent, rarely exceeding 120 beats to the minute until near the fatal close, when it often becomes imperceptible. Appetite is often well preserved, unless pneumonia or other complications supervene, but thirst is often intense, even though the tongue remain moist. The desire for food sometimes continues until within a few hours of death. Toward the last the skin becomes dry and cold, diarrhoea sets in, emaciation proceeds rapidly, collapse ensues and death.

Death usually occurs during the second week, often before the complete detachment of the eschar--in many instances by pneumonia, pulmonary gangrene, or entero-colitis. Some die in collapse, which is sometimes preceded by convulsions. When the eschars have become detached, suppuration exhausts the forces of the patient, and death takes place by asthenia.

The complication most frequent is pneumonia, and the next entero-colitis. Gangrene of the lungs, of the palate, pharynx, or oesophagus, of the anus, and of the vulva, may supervene. Hemorrhage from the facial artery or its branches has been noted as an exceptional mode of death (Hueber), the rule being that the arteries in the gangrenous area become plugged by thrombi, and thus prevent hemorrhage.

Recovery may take place before the local disease has penetrated the cheek--indeed, while the mucous membrane alone is involved. In recent instances, however, the disease does not subside until after the loss of considerable portions of the cheek, and the child recovers with great deformity, not only from loss of tissue in the cheek and nose, but from adhesions between the jaws and the cheek.

PATHOLOGY AND MORBID ANATOMY.--Gangrenous stomatitis always involves the cheek, almost always that portion in proximity to the mouth. It is almost invariably unilateral. Either side seems to be equally liable. Both sides suffer only, it is contended, when the gangrene is limited in extent, confined to the mucous membrane, and occupies the sides of the frenums of the lips (Barthez et Rilliet). It usually if not invariably begins in the mucous membrane, as a phlyctenular inflammation, which undergoes ulceration, followed by gangrene, immediately or not for several days, and then becomes covered with a more or less brownish-gray eschar. The ulceration of the mucous membrane is occasionally preceded by an oedematous condition of the cheek externally, similar to that sometimes observed in ordinary ulcerous stomatitis; but this is not the characteristic circumscribed, tense infiltration observed later. This ulceration is situated most frequently opposite the junction of the upper and lower teeth. Sometimes it proceeds from the gingivo-buccal sulcus of the lower jaw, sometimes from the alveolar border of the gums. It extends in all directions, and often reaches the lower lip. From three to sixteen days may be consumed in these extensions. The {342} surrounding mucous membrane becomes oedematous. The ulceration soon becomes followed by gangrene, sometimes within twenty-four hours, sometimes not for two or three days, and exceptionally not for several days. The ulcerated surfaces bleed readily, change from gray to black, and become covered with a semi-liquid or liquid putrescent detritus. They are sometimes surrounded by a projecting livid areola, which soon becomes gangrenous in its turn. The shreds of mortified membrane, though clinging a while to the sound tissues, are easily detached, and often drop spontaneously into the mouth. Meanwhile, there is abundant salivation, the products of which pour from the mouth, at first sanguinolent, and subsequently dark and putrescent and mixed with detritus of the tissues. Large portions of the gums, and even of the mucous membrane of the palate, may undergo destruction within a few (three to six) days. The gangrenous destruction of the gums soon exposes the teeth, which become loose and are sometimes spontaneously detached. Thence the periosteum and bone become implicated and undergo partial denudation and necrosis, and portions of necrosed bone become detached if the patient survives. The characteristic implication of the exterior of the cheek becomes manifest from the first to the third day, but occasionally not until a day or two later. A hard, circumscribed swelling of the cheek or cheek and lip occurs, sometimes preceded, as already intimated, by general oedematous infiltration. The surface is tense and unctuous, often discolored. In its central portion is an especially hard nucleus, one to two centimeters or more in diameter. Gangrene often takes place at this point from within outward at a period varying from the third to the seventh day or later. The skin becomes livid, then black; a pustule is formed at the summit of the swelling, which bursts and discloses a blackened gangrenous eschar from less than a line in thickness to the entire thickness of the cheek beneath. The area of gangrene gradually extends. The dead tissues become detached, and a perforation is left right through the cheek, through which are discharged saliva and detritus. Meanwhile, the submaxillary glands become swollen and the surrounding connective tissue becomes oedematous. In some instances, however, no change is noticeable in these glands.

Examinations after death have shown that thrombosis exists for some distance around the gangrenous mass. Hence the rarity of hemorrhage during the detachment of the eschar.

DIAGNOSIS.--In the early stage of the disease the main point of differential diagnosis rests in the locality of the primitive lesion, the mucous membrane of the inside of one cheek. Subsequently there is the gangrenous odor from the mouth; the rapid peripheric extension of the local lesion, which acquires a peculiar grayish-black color; its rapid extension toward the exterior of the cheek or lip; the tumefaction of the cheek, discolored, greasy, hard, surrounded by oedematous infiltration, and presenting a central nodule of especial hardness; then the profuse salivation, soon sanguinolent, subsequently purulent and mingled with detritus of the mortified tissues. Finally, the eschar on the exterior of the swollen cheek or lip leaves no doubt as to the character of the lesion. From malignant pustule it is distinguished by not beginning on the exterior, as that lesion always does (Baron).

PROGNOSIS.--The prognosis is bad unless the lesion be quite limited {343} and complications absent. At least three-fourths of those attacked perish; according to some authorities fully five-sixths die. The objective symptoms of the local disease are much more important in estimating the prognosis than are the constitutional manifestations, the vigor of the patient, and the hygienic surroundings, although, as a matter of course, the better these latter the more favorable the prognosis. Prognosis would be more favorable in private practice than in hospital or asylum service.

TREATMENT.--Active treatment is required, both locally and constitutionally. Local treatment is of paramount importance, and alone capable of arresting the extension of the process of mortification. The topical measure in greatest repute is energetic cauterization with the most powerful agents, chemical and mechanical--hydrochloric acid, nitric acid, acid solution of mercuric nitrate, and the actual cautery, whether hot iron, thermo-, or electric cautery. The application of acids is usually made with a firm wad or piece of sponge upon a stick or quill, care being taken to protect the healthy tissues as far as practicable with a spoon or spatula. After the application the mouth is to be thoroughly syringed with water to remove or dilute the superfluous acid. Hydrochloric acid has been preferred by most observers.

As these cauterizations must be energetic to prove effective, anæsthesia ought to be induced. Should ether be employed for this purpose, hydrochloric acid or the acid solution of mercuric nitrate would be selected of course.

In the early stages these agents are to be applied to the inside of the cheek, so as to destroy all the tissue diseased, if practicable, and expose a healthy surface for granulation. Should the exterior of the cheek become implicated before cauterization has been performed or in spite of it, it is customary to destroy the tissues from the exterior, including a zone of apparently healthy surrounding tissue. As the gangrene extends, the cauterization is to be repeated twice daily or even more frequently. After cauterization the parts are dressed with antiseptic lotions, and antiseptic injections or douches are to be used frequently during day and night to wash out the mouth and keep it as clear as possible from detritus.

Meigs and Pepper report beneficial results from the topical use of undiluted carbolic acid, followed by a solution of the same, one part in fifty of water, frequently employed as a mouth-wash. The progress of the sloughing was checked and the putridity of the unseparated dead tissue completely destroyed in the two cases mentioned by them, one of which recovered quickly without perforation of the cheek. Gerhard preferred undiluted tincture of the chloride of iron; Condie, cupric sulphate, thirty grains to the ounce. Bismuth subnitrate has recently been lauded as a topical remedial agent.[13]

[Footnote 13: Maguire, Medical Record N.Y., Feb. 3, 1883.]

The mouth should be frequently cleansed by syringing, douching, spraying, or washing with disinfectant solutions, such as chlorinated soda liquor, one part to ten; carbolic acid, one to twenty. Lemon-juice is sometimes an agreeable application, as in some other varieties of stomatitis. Constitutionally, tonic and supporting treatment is demanded, even in those instances where the appetite is well maintained and the {344} general health apparently well conserved. Soups, milk, semi-solid food, egg-nog, egg and wine, wine whey, milk punch, finely-minced meat, should be administered as freely as the state of the digestive functions will permit. If necessary, resort should be had to nutritive enemata. Quinia and tincture of chloride of iron are the medicines indicated. When sufficient alcohol cannot be given with the food, it should be freely exhibited in the most available form by the mouth or by the rectum. The apartment should be well ventilated, the linen frequently changed, the discharges promptly removed.

Toxic Stomatitis.

DEFINITION.--An inflammation of the interior of the mouth due to poisoning, especially by drugs, and chiefly by mercury, copper, and phosphorus.

Mercurial Stomatitis.

DEFINITION.--An inflammation of the mucous membrane of the mouth, eventually ulcerating, the result of systemic poisoning by the absorption of mercury.

SYNONYMS.--Stomatitis mercurialis; Mercurial ptyalism, Ptyalismus mercurialis; Mercurial salivation, Salivatio mercurialis.

ETIOLOGY--Predisposing and Exciting Causes.--Special vulnerability to the toxic influence of mercury, and special proclivity to inflammatory affections of the mouth and the organs contained therein, are the predisposing causes of mercurial stomatitis. The exciting cause is the absorption of mercury into the tissues of the organism. The susceptibility of healthy adults is much greater than that of healthy children. The susceptibility of adults varies very greatly. Constitutions deteriorated by prolonged disease, undue exposure, and the like are much more promptly influenced in consequence. Tuberculous subjects do not bear mercury well.

Idiosyncratic susceptibility to toxæmia by mercurial preparations is now and then encountered in practice, and instances have been published[14] in which fatal results have ensued, after prolonged suffering, from the incautious administration of a single moderate dose of a mercurial drug.

[Footnote 14: For example, see in Watson's Practice of Physic a case of furious salivation following one administration of two grains of calomel as a purgative, the patient dying at the end of two years, worn out by the effects of the mercury and having lost portions of the jaw-bone by necrosis.]

Until comparatively recent years the most common cause of mercurial poisoning was the excessive employment of mercurial medicines, whether by ingestion, inunction, or vapor bath. Topical cauterization with acid solution of mercuric nitrate is likewise an infrequent, and usually an accidental, cause of the affection. Elimination of the mercury by way of the mucous glands of mouth and the salivary glands proper excites the stomatitis in these instances. An entirely different series of cases occur in artisans exposed to handling the metal and its preparations or to breathing its vapor or its dust. In these instances the poison may gain {345} entrance into the absorbent system by the skin, the mucous membranes of the nose, mouth, and throat, the stomach, or the lungs. No matter what care may be exercised in cleansing the hands, it is often impossible to prevent occasional transference of the noxious material from fingers to throat, or to thoroughly free the finger-tips under the nails. The avocations entailing the risks of mercurial stomatitis comprise quicksilver-mining, ore-separating, barometer- and thermometer-making, gilding, hat-making, manufacturing of chemicals, and exhausting the globes employed in certain forms of electric illumination.[15] The slow absorption of mercury into the bodies of artisans induces in addition serious constitutional nervous disturbances--tremors, palsy, etc.

[Footnote 15: Med. and Surg. Reporter, Philada., Dec. 30, 1883, p. 734.]

SYMPTOMATOLOGY, COURSE, DURATION, TERMINATIONS, COMPLICATIONS, AND SEQUELÆ.--The principal subjective symptoms of mercurial stomatitis are--characteristic fetor of the breath, sore gums and mouth, continuous nauseous metallic brassy or coppery taste, and profuse salivation.

At first the mouth feels parched and painful, the gums tender, the teeth, the lower incisors especially, set on edge. Soon the gums become swollen, and when touched with the tongue seem to have receded from the necks of the teeth, which thereby appear to be longer than usual. The gums feel quite sore when pressed upon with the finger or when put on the stretch by clashing the rows of teeth against each other. This sort of soreness is often watched for in the therapeutic administration of mercurials purposely given to "touch the gums," as an indication that the system is under the influence of the drug. It is, therefore, one of the earliest indications of mercurial poisoning, but if not sought for it may elude attention until after the mouth has become sore a little later. The pain in the mouth is augmented by efforts of mastication and expectoration, and may be associated with pains at the angle of the lower jaw or extending along the domain of the third or of the third and second divisions of the distribution of the fifth cerebral nerve. Mastication of solid food is often unendurable. Constitutional manifestations become evident about this time in increased heat of skin, acceleration of pulse, furred tongue, dry mouth, great thirst, and loss of appetite. The dryness of the mouth does not last long, but is soon followed by hypersalivation, one of the characteristic phenomena of the disorder. The saliva secreted, often acid in reaction, varies greatly in quantity, which is usually proportionate to the severity of the case. It is secreted night and day, sometimes to the amount of several pints in the twenty-four hours--in moderately severe cases to the amount of from one to two pints in that space of time. It is limpid or grayish, mawkish or somewhat fetid, and reacts readily to the simplest tests for mercury. The salivation is almost continuous, sometimes quite so. The patient soon becomes unable to endure the fatigue of constant expectoration, and the fluid then dribbles from his mouth or runs off in an unimpeded slobber. When excessive, the patient's strength becomes rapidly exhausted--in part by impoverishment of the fluids, in great measure from the lack of refreshing sleep.

Meanwhile, the local inflammatory process extends from the gums to the floor of the mouth and to the lips, and thence to the tongue and the {346} cheeks. The salivary glands are in a state of inflammation likewise, but rather in consequence of direct irritation in the elimination of the poison through their channels than by extension of the stomatitis along their ducts. The lymphatic glands of the lower jaw become engorged and tender. Mastication, deglutition, and articulation all become impeded mechanically by tumefaction of the tissues.

In some instances the glossitis is so great that the tongue protrudes, thereby impeding respiration and even threatening suffocation. In some cases oedema of the larynx has been noted, threatening suffocation from that cause. Should the inflammatory process extend along the pharynx to the Eustachian tubes, deafness and pains in the ears will become additional symptoms.

The subsequent progress of unarrested mercurial stomatitis is that of ulcerous stomatitis.

Should gangrene of the mucous membrane take place, there will be great fetor from the mouth, and some danger of hemorrhage on detachment of the sloughs should the process be taking place in the direction of vessels of some calibre. Necrosis of the inferior maxilla entails continuance of the disagreeable local symptoms until the discharge in fragments or in mass of the dead portions of bone.

In the earlier stages of the attack the constitutional symptoms may be sthenic. Fever, cephalalgia, and the usual concomitants of pyrexia, however, soon give way to the opposite condition of asthenia. Exhausted by the excessive salivation, and unable to repair waste by eating or sleeping, the sufferer soon passes into a condition of hopeless cachexia. Those who survive remain cachectic and feeble for a long time--some of them disfigured for life by various cicatrices between cheeks and jaw, by loss of teeth or of portions of the jaw-bone.

The duration of mercurial stomatitis varies with the susceptibility of the patient, the intensity of the toxæmia, and the character of the treatment. Mild cases may get well in a week or two; severe cases may continue for weeks, and even months; extreme cases have persisted for years. Even moderate cases occasionally resist treatment for weeks.

Under the improved therapeutics of the present day mercurial stomatitis almost always terminates in recovery, especially if it receive early and prompt attention. Neglected or improperly managed, it may terminate in serious losses of tissue in gums, cheeks, teeth, and bone, leaving the parts much deformed and the patient in a permanently enfeebled condition.

Erysipelas, metastatic abscesses, inflammations, pyæmia, or colliquative diarrhoea may be mentioned as complications which may prove sufficiently serious to produce death, independently of the virulence of the primary stomatitis.

PATHOLOGY AND MORBID ANATOMY.--Mercurial stomatitis is an ulcerative process attended with an excessive flow of saliva containing mercury. It has a tendency to terminate in destruction and exfoliation of the mucous membrane of the gums and other tissues attacked, and eventually in necrosis of the jaw-bone. The detritus is found, microscopically, to consist of granular masses of broken-down tissue, swarming with bacteria and micrococci, and containing some blood-cells and many pus-cells. In some instances micrococci have been detected in the blood.

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