Anomalies And Curiosities Of Medicine Part 63

Diffuse symmetric scleroderma, or hide-bound disease, is quite rare, and presents itself in two phases: that of infiltration (more properly called hypertrophy) and atrophy, caused by shrinkage. The whole body may be involved, and each joint may be fixed as the skin over it becomes rigid. The muscles may be implicated independently of the skin, or simultaneously, and they give the resemblance of rigor mortis. The whole skin is so hard as to suggest the idea of a frozen corpse, without the coldness, the temperature being only slightly subnormal.

The skin can neither be pitted nor pinched. As Crocker has well put it, when the face is affected it is gorgonized, so to speak, both to the eye and to the touch. The mouth cannot be opened; the lids usually escape, but if involved they are half closed, and in either case immovable. The effect of the disease on the chest-walls is to seriously interfere with the respiration and to flatten and almost obliterate the b.r.e.a.s.t.s; as to the limbs, from the shortening of the distended skin the joints are fixed in a more or less rigid position. The mucous membranes may be affected, and the secretion of both sweat and seb.u.m is diminished in proportion to the degree of the affection, and may be quite absent. The atrophic type of scleroderma is preceded by an edema, and from pressure-atrophy of the fat and muscles the skin of the face is strained over the bones; the lips are shortened, the gums shrink from the teeth and lead to caries, and the nostrils are compressed. The strained skin and the emotionless features (relieved only by telangiectatic striae) give the countenance a ghastly, corpse-like aspect. The etiology and pathology of this disease are quite obscure.

Happily the prognosis is good, as there is a tendency to spontaneous recovery, although the convalescence may be extended.

Although regarded by many as a disease distinct from scleroderma, morphea is best described as a circ.u.mscribed scleroderma, and presents itself in two clinical aspects: patches and bands, the patches being the more common.

Scleroderma neonatorum is an induration of the skin, congenital and occurring soon after birth, and is invariably fatal. A disease somewhat a.n.a.logous is edema neonatorum, which is a subcutaneous edema with induration affecting the new-born. If complete it is invariably fatal, but in a few cases in which the process has been incomplete recovery has occurred. Gerard reports recovery from a case of sclerema neonatorum in an infant five weeks old, which seemed in perfect health but for this skin-affection. The back presented a remarkable induration which involved the entire dorsal aspect, including the deltoid regions, the upper arms, the b.u.t.tocks, and the thighs, down to and involving the popliteal s.p.a.ces. The edges of the indurated skin were sharply defined, irregular, and map-like. The affected skin was stretched, but not s.h.i.+ny, and exhibited a pink mottling; it could not be pinched between the fingers; pressure produced no pitting, but rendered the surface pale for a time. The induration upon the b.u.t.tocks had been noticed immediately after birth, and the region was at first of a deep pink color. During the first nine days the trouble had extended to the thighs, but only shortly before the examination had it attacked the arms. Inunctions of codliver oil were at first used, but with little improvement. Blue ointment was subst.i.tuted, and improvement commenced.

As the induration cleared up, outlying patches of the affected skin were left surrounded by normal integument. No pitting could be produced even after the tension of the skin had decreased during recovery. The lowest rectal temperature was 98 degrees F. In a little more than four months the skin became normal. The treatment with mercurial ointment was stopped some time before recovery.

Possibly the most interesting of the examples of skin-anomaly was the "elephant-man" of London. His real name was Merrick. He was born at Leicester, and gave an elaborate account of shock experienced by his mother shortly before his birth, when she was knocked down by an elephant at a circus; to this circ.u.mstance he attributed his unfortunate condition. He derived his name from a proboscis-like projection of his nose and lips, together with a peculiar deformity of the forehead. He was victimized by showmen during his early life, and for a time was shown in Whitechapel Road, where his exhibition was stopped by the police. He was afterward shown in Belgium, and was there plundered of all his savings. The gruesome spectacle he presented ostracized him from the pleasures of friends.h.i.+p and society, and sometimes interfered with his travels. On one occasion a steamboat captain refused to take him as a pa.s.senger. Treves exhibited him twice before the Pathological Society of London. His affection was not elephantiasis, but a complication of congenital hypertrophy of certain bones and pachydermatocele and papilloma of the skin. From his youth he suffered from a disease of the left hip-joint. The papillary ma.s.ses developed on the skin of the back, b.u.t.tock, and occiput. In the right pectoral and posterior aspect of the right axillary region, and over the b.u.t.tocks, the affected skin hung in heavy pendulous flaps. His left arm was free from disease. His head grew so heavy that at length he had great difficulty in holding it up. He slept in a sitting or crouching position, with his hands clasped over his legs, and his head on his knees. If he lay down flat, the heavy head showed a tendency to fall back and produce a sense of suffocation. For a long time he was an inmate of the London Hospital, where special quarters were provided for him, and it was there that he was found dead, April 11, 1890; while in bed his ponderous head had fallen backward and dislocated his neck.

Ainhum may be defined as a pathologic process, the ultimate result of which is a spontaneous amputation of the little toe. It is confined almost exclusively to negroes, chiefly males, and of African descent.

In Brazil it is called "ainham" or "quigila." "Ainham" literally means to saw, and is doubtless a colloquial name derived from a supposed slow, sawing process. The Hindoo name for it is "sukha pakla," meaning dry suppuration.

In 1866 da Silva Lima of Bahia, at the Misericordia Hospital, gave the first reports of this curious disease, and for quite a period it was supposed to be confined to Brazilian territory. Since then, however, it has been reported from nearly every quarter of the globe. Relative to its geographic distribution, Pyle states that da Silva Lima and Seixas of Bahia have reported numerous cases in Brazil, as have Figueredo, Pereira, Pirovano, Alpin, and Guimares. Toppin reports it in Pernambuco. Mr. Milton reports a case from Cairo, and Dr. Creswell at Suez, both in slaves. E. A. G. Doyle reports several cases at the Fernando Hospital, Trinidad. Digby reports its prevalence on the west coast of Africa, particularly among a race of negroes called Krumens.

Messum reports it in the South African Republic, and speaks of its prevalence among the Kaffirs. Eyles reports it on the Gold Coast. It has also been seen in Algiers and Madagascar. Through the able efforts of Her Majesty's surgeons in India the presence of ainhum has been shown in India, and considerable investigation made as to its etiology, pathologic histology, etc. Wise at Dacca, Smyth and Crombie at Calcutta, Henderson at Bombay, and Warden, Sen, Crawford, and Cooper in other portions of Southern India have all rendered a.s.sistance in the investigation of ainhum. In China a case has been seen, and British surgeons speak of it as occurring in Ceylon. Von Winckler presents an admirable report of 20 cases at Georgetown, British Guiana. Dr.

Potoppidan sends a report of a case in a negress on St. Thomas Island.

The disease has several times been observed in Polynesia.

Dr. Hornaday reports a case in a negress from North Carolina, and, curious to relate, Horwitz of Philadelphia and Shepherd of Canada found cases in negroes both of North Carolina antecedents. Dr. James Evans reports a case in a negro seventy-four years of age, at Darlington, S.C. Dr. R. H. Days of Baton Rouge, La., had a case in a negress, and Dr. J. L. Deslates, also of Louisiana, reports four cases in St. James Parish. Pyle has seen a case in a negress aged fifty years, at the Emergency Hospital in Was.h.i.+ngton.

So prevalent is the disease in India that Crawford found a case in every 2500 surgical cases at the Indian hospitals. The absence of pain or inconvenience in many instances doubtless keeps the number of cases reported few, and again we must take into consideration the fact that the cla.s.s of persons afflicted with ainhum are seldom brought in contact with medical men.

The disease usually affects the 5th phalanx at the interphalangeal joint. Cases of the 4th and other phalanges have been reported. Cooper speaks of a young Brahman who lost his left great toe by this process.

Crombie speaks of a simultaneous amputation of both fourth toes.

Potoppidan reports a similar case in a negress on St. Thomas Island.

Sen reports a case in a supernumerary digit in a child, whose father, a Hindoo, lost a toe by ainhum. Eyles reports a case in a negro in whom the second finger was affected. Mirault, at Angiers, speaks of a case in which two fingers were lost in fifteen days, a fact which makes his diagnosis dubious. Beranger-Ferraud has seen all the toes amputated, and there is a wax model by Baretta, Paris, in the Army Medical Museum at Was.h.i.+ngton, in which all the toes of the right foot have been amputated, and the process is fast making progress at the middle third of the leg.

Ainhum is much more common in males than in females; it is, in fact, distinctly rare in the latter. Of von Winckler's 20 cases all were males.

It may occur at any age, but is most common between thirty and thirty-five. It has been reported in utero by Guyot, and was seen to extend up to the thigh, a statement that is most likely fallacious.

However, there are well-authenticated cases in infants, and again in persons over seventy years of age.

In some few cases the metatarso-phalangeal joint is affected; but no case has been seen at the base of the ungual phalanx. The duration of the disease is between two and four years, but Dr. Evans's case had been in progress fifty years. It rarely runs its full course before a year.

Ainhum begins as a small furrow or crack, such as soldiers often experience, at the digito-plantar fold, seen first on the inner side.

This process of furrowing never advances in soldiers, and has been given a name more expressive than elegant. In ainhum the toe will swell in a few days, and a pain, burning or shooting in nature, may be experienced in the foot and leg affected. Pain, however, is not constant. There may be an erythematous eruption accompanying the swelling. The furrow increases laterally and in depth, and meets on the dorsal aspect of the toe, giving the toe the appearance of being constricted by a piece of fine cord. As the furrow deepens the distal end of the toe becomes ovoid, and soon an appearance as of a marble attached to the toe by a fibrous pedicle presents itself. By this time the swelling, if any, has subsided. The distal end of the toe bends under the foot, and becomes twisted when walking, and causes inconvenience, and, unfortunately, says Eyles, it is in this last stage only that the Fanti presents himself. There is in the majority of cases a small ulcer in or near the digito-plantar fold, which causes most of the pain, particularly when pressed upon. This ulcer does not occur early, and is not constant. The case under Pyle's observation showed no ulceration, and was absolutely painless, the negress applying for diagnosis rather than treatment. The furrow deepens until spontaneous amputation takes place, which rarely occurs, the patient generally hastening the process by his own operation, or by seeking surgical treatment. A dry scab forms at the furrow, and when picked and repicked constantly re-forms, being composed of h.o.r.n.y desquamation or necrosis.

The histology of ainhum shows it to be a direct ingrowth of epithelium, with a corresponding depression of surface due to a rapid hyperplasia that pushes down and strangles the papillae, thus cutting off the blood supply from the epithelial cells, causing them to undergo a h.o.r.n.y change.

The disease is not usually symmetric, as formerly stated, nor is it simultaneous in different toes. There are no a.s.sociated const.i.tutional symptoms, no tendency to similar morbid changes in other parts, and no infiltration elsewhere. There is little or no edema with ainhum. In ainhum there is, first, simple hypertrophy, then active hyperplasia The papillae degenerate when deprived of blood supply, and become h.o.r.n.y.

Meanwhile the pressure thus exerted on the nervi vasorum sets up vascular changes which bring about epithelial changes in more distant areas, the process advancing anteriorly, that is, in the direction of the arteries. This makes the cause, according to Eyles, an inflammatory and trophic phenomenon due mainly to changes following pressure on the vasomotor nerves.

Etiology.--The theories of the causation of ainhum are quite numerous.

The first cause is the admirable location for a furrow in the digito-plantar fold, and the excellent situation of the furrow for the entrance of sand or other particles to make the irritation constant, thus causing chronic inflammatory changes, which are followed subsequently by the changes peculiar to ainhum. The cause has been ascribed to the practice of wearing rings on the toes; but von Winckler says that in his locality (British Guinea) this practice is confined to the coolie women, and in not one of his 20 cases had a ring been previously worn on the toe; in fact all of the patients were males.

Digby says, however, that the Krumens, among whom the disease is common, have long worn bra.s.s or copper rings on the fifth toe. Again the natives of India, who are among those most frequently afflicted, have no such custom.

Injury, such as stone-bruise, has been attributed as the initial cause, and well-authenticated cases have been reported in which traumatism is distinctly remembered; but Smyth, Weber, and several other observers deny that habits, accidents, or work, are a feature in causation.

Von During reports a curious case which he calls sclerodactylia annularis ainhumoides. The patient was a boy about twelve years old, born in Erzeroum, brought for treatment for scabies, and not for the affection about to be described. A very defective history led to the belief that a similar affection had not been observed in the family.

When he was six years old it began on the terminal phalanges of the middle fingers. A myxomatous swelling attacked the phalanges and effected a complete absorption of the terminal phalanx. It did not advance as far as gangrene or exfoliation of bone. At the time of report the whole ten fingers were involved; the bones seemed to be thickened, the soft parts being indurated or sclerosed. In the right index finger a completely sclerosed ring pa.s.sed around the middle phalanx. The nails on the absorbed phalanges had become small and considerably thickened plates. No a.n.a.logous changes were found elsewhere, and sensation was perfectly normal in the affected parts.

There were no signs whatever of a multiple neuritis nor of a leprous condition.

There is a rare and curious condition known as "deciduous skin" or keratolysis, in which the owners possess a skin, which, like that of a serpent, is periodically cast off, that of the limbs coming off like the finger of a glove. Preston of Canterbury, New Zealand, mentions the case of a woman who had thus shed her skin every few weeks from the age of seven or even earlier. The woman was sixty-seven years of age; the skin in every part of the body came away in casts and cuticles which separated entire and sometimes in one unbroken piece like a glove or stocking. Before each paroxysm she had an a.s.sociate symptom of malaise.

Even the skin of the nose and ears came off complete. None of the patient's large family showed this idiosyncrasy, and she said that she had been told by a medical man that it had been due to catching cold after an attack of small-pox. Frank mentions a case in which there was periodic and complete shedding of the cuticle and nails of the hands and feet, which was repeated for thirty-three consecutive years on July 24th of each year, and between the hours of 3 P.M. and 9 P.M. The patient remembered shedding for the first time while a child at play.

The paroxysms always commenced abruptly, const.i.tutional febrile symptoms were first experienced, and the skin became dry and hot. The acute symptoms subsided in three or four hours and were entirely gone in twelve hours, with the exception of the redness of the skin, which did not disappear for thirty-six hours more. The patient had been delirious during this period. The cuticle began to shed some time between the third and twelfth day, in large sheets, as pictured in the accompanying ill.u.s.trations. The nails were shed in about four weeks after the acute stage. Crocker had an instance of this nature in a man with tylosis palmae, in which the skin was cast off every autumn, but the process lasted two months. Lang observed a case in which the fingers alone were affected.

There is a case of general and habitual desquamation of the skin in the Ephemerides of 1686; and Newell records a case which recovered under the use of Cheltenham water for several seasons. Latham describes a man of fifty who was first seized about ten years previously with a singular kind of fever, and this returned many times afterward, even twice in the course of the same year, attended with the same symptoms and circ.u.mstances, and appearing to be brought on by obstructed perspiration, in consequence of catching cold. Besides the common febrile symptoms, upon the invasion of the disease his skin universally itched, more especially at the joints, and the itching was followed by many little red spots, with a small degree of swelling. Soon after this his fingers became stiff; hard, and painful at the ends, and at the roots of the nails. In about twenty-four hours the cuticle began to separate from the cutis, and in ten or twelve days this separation was general from head to foot, during which time he completely turned the cuticle off from the wrists to the fingers' ends like a glove, and in like manner on the legs to the toes, after which his nails shot gradually from their roots, at first with exquisite pain, which abated as the separation of the cuticle advanced, and the old nails were generally thrown off by new ones in about six months. The cuticle rose in the palms and soles like blisters, having, however, no fluid beneath, and when it came off it left the underlying cutis exposed for a few days. Sometimes, upon catching cold, before quite free from feverish symptoms, a second separation of the cuticle from the cutis occurred, but it appeared so thin as to be like scurf, demonstrating the quick renewal of the parts.

There is a similar case in the Philosophical Transactions in a miller of thirty-five who was exposed to great heat and clouds of dust. On the first cold a fever attacked him, and once or twice a year, chiefly in the autumn, this again occurred, attended with a loosening and detachment of the cuticle. The disorder began with violent fever, attended with pains in the head, back, limbs, retching, vomiting, dry skin, furred tongue, urgent thirst, constipation, and high-colored urine. Usually the whole surface of the body then became yellow. It afterward became florid like a rash, and then great uneasiness was felt for several days, with general numbness and tingling; the urine then began to deposit a thick sediment. About the third week from the first attack the cuticle appeared elevated in many places, and in eight or ten days afterward became so loose as to admit of its easy removal in large flakes. The cuticle of the hands, from the wrists to the fingers'

ends, came off like a glove. The patient was never disposed to sweat, and when it was attempted to force perspiration he grew worse; nor was he much at ease until his urine deposited a sediment, after which he felt little inconvenience but from the rigidity of the skin. The nails were not detached as in the previous case.

It is quite natural that such cases as this should attract the attention of the laity, and often find report in newspapers. The following is a lay-report of a "snake-boy" in Shepardstown, Va.:--

"Jim Twyman, a colored boy living with his foster-parents ten miles from this place, is a wonder. He is popularly known as the "snake-boy."

Mentally he is as bright as any child of his age, and he is popular with his playmates, but his physical peculiarities are probably unparalleled. His entire skin, except the face and hands, is covered with the scales and markings of a snake. These exceptions are kept so by the constant use of Castile soap, but on the balance of his body the scales grow abundantly. The child sheds his skin every year. It causes him no pain or illness. From the limbs it can be pulled in perfect shape, but off the body it comes in pieces. His feet and hands are always cold and clammy. He is an inordinate eater, sometimes spending an hour at a meal, eating voraciously all the time, if permitted to do so. After these gorgings he sometimes sleeps two days. There is a strange suggestion of a snake in his face, and he can manipulate his tongue, accompanied by hideous hisses, as viciously as a serpent."

Under the name of dermat.i.tis exfoliativa neonatorum, Ritter has described an eruption which he observed in the foundling asylum at Prague, where nearly 300 cases occurred in ten years. According to Crocker it begins in the second or third week of life, and occasionally as late as the fifth week, with diffuse and universal scaling, which may be branny or in laminae like pityriasis rubra, and either dry or with suffusion beneath the epidermis. Sometimes it presents flaccid bullae like pemphigus foliaceus, and then there are crusts as well as scales, with rhagades on the mouth, a.n.u.s, etc.; there is a total absence of fever or other general symptoms. About 50 per cent die of marasmus and loss of heat, with or without diarrhea. In those who recover the surface gradually becomes pale and the desquamation ceases.

Opinions differ regarding it, some considering it of septic origin, while others believe it to be nothing but pemphigus foliaceus. Kaposi regards it as an aggravation of the physiologic exfoliation of the new-born. Elliott of New York reports two cases with a review of the subject, but none have been reported in England. Cases on the Continent have been described by Billard, von Baer, Caspary, those already mentioned, and others.

The name epidemic exfoliative dermat.i.tis has been given to an epidemic skin-disease which made its appearance in 1891 in England; 425 cases were collected in six inst.i.tutions, besides sporadic cases in private houses.

In 1895, in London, some photographs and sketches were exhibited that were taken from several of the 163 cases which occurred in the Paddington Infirmary and Workhouse, under the care of Dr. Savill, from whose negatives they were prepared. They were arranged in order to ill.u.s.trate the successive stages of the disorder. The eruption starts usually with discrete papules, often in stellate groups, and generally arranged symmetrically when on the limbs. These become fused into crimson, slightly raised maculae, which in severe cases become further fused into red thickened patches, in which the papules can still be felt and sometimes seen. Vesicles form, and exudation occurs in only about one-third of the cases. Desquamation of the epidermis is the invariable feature of all cases, and it usually commences between the fourth and eighth days. In severe cases successive layers of the epidermis are shed, in larger or smaller scales, throughout the whole course of the malady. One-half of the epidermis shed from the hand of a patient is exhibited in this collection.

Of sphaceloderma, or gangrene of the skin, probably the most interesting is Raynaud's disease of symmetric gangrene, a vascular disorder, which is seen in three grades of intensity: there is local syncope, producing the condition known as dead-fingers or dead-toes, and a.n.a.logous to that produced by intense cold; and local asphyxia, which usually follows local syncope, or may develop independently.

Chilblains are the mildest manifestation of this condition. The fingers, toes, and ears, are the parts usually affected. In the most extreme degree the parts are swollen, stiff, and livid, and the capillary circulation is almost stagnant; this is local or symmetric gangrene, the mildest form of which follows asphyxia. Small areas of necrosis appear on the pads of the fingers and of the toes; also at the edges of the ears and tip of the nose. Occasional symmetric patches appear on the limbs and trunk, and in extensive cases terminate in gangrene. Raynaud suggested that the local syncope was produced by contraction of the vessels; the asphyxia is probably caused by a dilatation of the capillaries and venules, with persistence of the spasm of the arterioles. According to Osler two forms of congestion occur, which may be seen in adjacent fingers, one of which may be swollen, intensely red, and extremely hot; the other swollen, cyanotic, and intensely cold. Sometimes all four extremities are involved, as in Southey's case, in a girl of two and a half in whom the process began on the calves, after a slight feverish attack, and then numerous patches rapidly becoming gangrenous appeared on the backs of the legs, thighs, b.u.t.tocks, and upper arms, worse where there was pressure; the child died thirty-two hours after the onset. The whole phenomenon may be unilateral, as in Smith's case, quoted by Crocker,--in a girl of three years in whom the left hand was cold and livid, while on the right there was lividity, progressing to gangrene of the fingers and of the thumb up to the first knuckles, where complete separation occurred.

A considerable number of cases of apparently spontaneous gangrene of the skin have been recorded in medical literature as occurring generally in hysteric young women. Crocker remarks that they are generally cla.s.sified as erythema gangraenosum, and are always to be regarded with grave suspicion of being self-induced. Ehrl records an interesting case of this nature with an accompanying ill.u.s.tration. The patient was a girl of eighteen whose face, left breast, a.n.u.s, legs, and feet became affected every autumn since her sixth year, after an attack of measles. At first the skin became red, then water-blisters formed, the size of a grain of corn, and in three days reaching the size of a hazel-nut; these burst and healed, leaving no scars. The menses appeared at the fifteenth year, lasted eight days, with great loss of blood, but there was no subsequent menstruation, and no vicarious hemorrhage. Afterward the right half of the face became red for three or four weeks, with a disturbance of the sensibility of this part, including the right half of the mucosa of the mouth and the conjunctive of the right eye. At the seventeenth year the patient began to have a left-sided headache and increased sweating of the right half of the body. In 1892 the periodically-appearing skin-affection became worse.

Instead of healing, the broken vessels became blackish and healed slowly, leaving ulcers, granulations, and scars, and the gangrenous tendency of the skin increased. Disturbance of the sight shortly intervened, a.s.sociated with aphonia. The sensibility of the whole body, with the exception of the face, was greatly impaired, and there was true gangrene of the corium. A younger sister of the patient was similarly affected with symptoms of hysteria, hemianesthesia, etc.

Neuroses of the skin consist in augmentation of sensibility or hyperesthesia and diminution of sensibility or anesthesia. There are some curious old cases of loss of sensation. Ferdinandus mentions a case of a young man of twenty-four who, after having been seized with insensibility of the whole body with the exception of the head, was cured by purgatives and other remedies. Bartholinus cites the case of a young man who lost the senses of taste and feeling; and also the case of a young girl who could permit the skin of her forehead to be p.r.i.c.ked and the skin of her neck to be burned without experiencing any pain. In his "Surgery" Lamothe mentions a case of insensibility of the hands and feet in consequence of a horse-kick in the head without the infliction of any external wound. In the "Memoires de l'Academie des Sciences" for the year 1743, we read an account of a soldier who, after having accidentally lost all sensation in his left arm, continued to go through the whole of the manual exercise with the same facility as ever. It was also known that La Condamine was able to use his hands for many years after they had lost their sensation. Rayer gives a case of paralysis of the skin of the left side of the trunk without any affection of the muscles, in a man of forty-three of apoplectic const.i.tution. The paralysis extended from the left mammary region to the haunch, and from the vertebrae to the linea alba. Throughout this whole extent the skin was insensible and could be pinched or even punctured without the patient being aware that he was even touched. The parts did not present any perceptible alteration in texture or in color. The patient was free from fever and made no complaint except a slight headache. Rayer quotes another case in a man of sixty who had been bitten three years previously by a dog that was not mad. He was greatly frightened by the accident and every time he saw a dog he trembled violently, and on one occasion he suffered a convulsive attack for one and a half hours. The convulsions increased in number and frequency, he lost his memory, and exhibited other signs of incipient dementia. He was admitted to the hospital with two small wounds upon the head, one above the left eyebrow and the other on the scalp, occasioned by a fall on his entrance into the hospital. For several days a great degree of insensibility of the skin of the whole body was observed without any implication of the power of voluntary motion. He was entirely cured in eighteen days.

Duhring reports a very rare form of disease of the skin, which may be designated neuroma cutis dolorosum, or painful neuroma of the skin. The patient was a boiler-maker of seventy who had no family history bearing on the disease. Ten years previously a few cutaneous tubercles the size and shape of a split-pea were noticed on the left shoulder, attended with decided itching but not with pain. The latter symptom did not come on until three years later. In the course of a year or two the lesions increased in number, so that in four years the shoulder and arm were thickly studded with them. During the next five years no particular changes occurred either in lesions or in the degree of pain. The region affected simply looked like a solid sheet of variously-sized, closely-packed, confluent tubercles, hard and dense. The tubercles were at all times painful to the touch, and even the contact of air was sufficient to cause great suffering. During the paroxysms, which occurred usually at several short intervals every day, the skin changed color frequently and rapidly, pa.s.sing through various reddish and violet tints, at times becoming purplish.

As a paroxysm came on the man was in the habit of gently pressing and holding the arm closely to his body. At one time he endured the attack in a standing posture, walking the floor, but usually he seated himself very near a hot stove, in a doubled-up, cramped position, utterly unmindful of all surroundings, until the worst pain had ceased.

Frequently he was unable to control himself, calling out piteously and vehemently and beseeching that his life be terminated by any means. In desperation he often lay and writhed on the floor in agony. The intense suffering lasted, as a rule, for about a half hour, but he was never without pain of the neuralgic type. He was freer of pain in summer than in winter. Exsection of the brachial plexus was performed, but gave only temporary relief. The man died in his eighty-fourth year of senile debility.

According to Osler the tubercula dolorosa or true fascicular neuroma is not always made up of nerve-fibers, but, as shown by Hoggan, may be an adenomatous growth of the sweat-glands.

Yaws may be defined as an endemic, specific, and contagious disease, characterized by raspberry-like nodules with or without const.i.tutional disturbance. Its synonym, frambesia, is from the French, framboise, a raspberry. Yaws is derived from a Carib word, the meaning of which is doubtful. It is a disease confined chiefly to tropical climates, and is found on the west coast of Africa for about ten degrees on each side of the equator, and also on the east coast in the central regions, but rarely in the north. It is also found in Madagascar, Mozambique, Ceylon, Hindoostan, and nearly all the tropical islands of the world.

Crocker believes it probable that the b.u.t.ton-scurvy of Ireland, now extinct, but described by various writers of 1823 to 1857 as a contagious disease which was prevalent in the south and in the interior of the island, was closely allied to yaws, if not identical with it.

The first mention of the yaws disease is by Oviedo, in 1535, who met with it in San Domingo. Although Sauvages at the end of the last century was the first to give an accurate description of this disease, many physicians had observed it before.

Frambesia or yaws was observed in Brazil as early as 1643, and in America later by Lebat in 1722. In the last century Winterbottom and Hume describe yaws in Africa, Hume calling it the African distemper. In 1769 in an essay on the "Natural History of Guiana," Bancroft mentions yaws; and Thomson speaks of it in Jamaica. Hillary in 1759 describes yaws in Barbadoes; and Bajou in Domingo and Cayenne in 1777, Dazille having already observed it in San Domingo in 1742.

Crocker takes his account of yaws from Numa Rat of the Leeward Islands, who divides the case into four stages: incubation, primary, secondary, and tertiary. The incubation stage is taken from the date of infection to the first appearance of the local lesion at the sight of inoculation. It varies from three to ten weeks. The symptoms are vague, possibly palpitation, vertigo, edema of the limbs and eyelids. The primary stage begins with the initial lesion, which consists of a papule which may be found most anywhere on the body. This papule ulcerates. The secondary stage commences about a fortnight after the papule has healed. There is intermittent fever, headache, backache, and shooting pains in the limbs and intercostal s.p.a.ces, like those of dengue, with nocturnal exacerbations. An eruption of minute red spots appears first on the face, and gradually extends so that the whole body is covered at the end of three days. By the seventh day the apex of the papule is of a pale yellow color, and the black skin has the appearance of being dotted over with yellow wax. The papule then develops into nodules of cylindric shape, with a dome-shaped, thick, yellow crust. It is only with the crust off that there is any resemblance to a raspberry. During the month following the raspberry appearance the skin is covered with scabs which, falling off, leave a pale macula; in dark races the macula becomes darker than normal, but in pale races it becomes paler than the natural skin, and in neither case is it scarcely ever obliterated. Intense itching is almost always present, and anemia is also a constant symptom. The disease is essentially contagious and occurs at all ages and among all s.e.xes, to a lesser degree in whites and hybrids, and is never congenital. It seems to have a tendency to undergo spontaneous recovery.

Furunculus orientalis, or its synonyms, Oriental boil, Aleppo boil, Delhi boil, Biskra b.u.t.ton, etc., is a local disease occurring chiefly on the face and other uncovered spots, endemic in limited districts in hot climates, characterized by the formation of a papule, a nodule, and a scab, and beneath the last a sharply punched-out ulcer. Its different names indicate the districts in which it is common, nearly always in tropical or subtropical climates. It differs from yaws in the absence of febrile symptoms, in its unity, its occurrence often on the feet and the backs of the hands, its duration, and the deep scar which it leaves. A fatal issue is rare, but disfiguring and disabling cicatrices may be left unless great care is employed.