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Among the older medical writers Weber speaks of singultus lasting for five days; Tulpius, for twelve days; Eller and Schenck, for three months; Taranget, for eight months; and Bartholinus, for four years.
At the present day it is not uncommon to read in the newspapers accounts of prolonged hiccoughing. These cases are not mythical, and are paralleled by a number of instances in reliable medical literature. The cause is not always discernible, and cases sometimes resist all treatment.
Holston reports a case of chronic singultus of seven years' standing. It had followed an attack of whooping-cough, and was finally cured apparently by the administration of strychnin. Cowan speaks of a shoemaker of twenty-two who experienced an attack of constant singultus for a week, and then intermittent attacks for six years. Cowan also mentions instances of prolonged hiccough related by Heberden, Good, Hoffman, and Wartmouth. Barrett is accredited with reporting a case of persistent hiccough in a man of thirty-five. Rowland speaks of a man of thirty-five who hiccoughed for twelve years. The paroxysms were almost constant, and occurred once or twice a minute during the hours when the man was not sleeping. There was no noise with the cough. There is another case related in the same journal of a man who died on the fourth day of an attack of singultus, probably due to abscess of the diaphragm, which no remedy would relieve. Moore records a case of a child, injured when young, who hiccoughed until about twenty years of age (the age at the time of report). Foot mentions a lad of fifteen who, except when asleep, hiccoughed incessantly for twenty-two weeks, and who suffered two similar, but less severe, attacks in the summer of 1879, and again in 1880. The disease was supposed to be due to the habit of pressing the chest against the desk when at school. Dexter reports a case of long-continued singultus in an Irish girl of eighteen, ascribed to habitual masturbation. There was no intermission in the paroxysm, which increased in force until general convulsions ensued. The patient said that the paroxysm could be stopped by firm pressure on the upper part of the external genital organs. Dexter applied firm pressure on her clitoris, and the convulsions subsided, and the patient fell asleep. They could be excited by firm pressure on the lower vertebrae. Corson speaks of a man of fifty-seven who, after exposure to cold, suffered exhausting hiccough for nine days; and also records the case of an Irish servant who suffered hiccough for four months; the cause was ascribed to fright. Stevenson cites a fatal instance of hiccough in a stone-mason of forty-four who suffered continuously from May 14th to May 28th. The only remedy that seemed to have any effect in this case was castor-oil in strong purgative doses.
Willard speaks of a man of thirty-four who began to hiccough after an attack of pneumonia, and continued for eighty-six hours. The treatment consisted of the application of belladonna and cantharides plasters, bismuth, and lime-water, camphor, and salts of white hellebore inhaled through the nose in finest powder. Two other cases are mentioned by the same author. Gapper describes the case of a young man who was seized with loud and distressing hiccough that never ceased for a minute during eighty hours. Two ounces of laudanum were administered in the three days without any decided effect, producing only slight languor.
Ranney reports the case of an unmarried woman of forty-four who suffered from paroxysms of hiccough that persisted for four years. A peculiarity of this attack was that it invariably followed movements of the upper extremities. Tenderness and hyperesthesia over the spinous processes of the 4th, 5th, and 6th cervical vertebrae led to the application of the thermocautery, which, in conjunction with the administration of ergot and bromide, was attended with marked benefit, though not by complete cure. Barlow mentions a man with a rheumatic affection of the shoulder who hiccoughed when he moved his joints. Barlow also recites a case of hiccough which was caused by pressure on the cicatrix of a wound in the left hand.
Beilby reports a peculiar case in a girl of seventeen who suffered an anomalous affection of the respiratory muscle, producing a sound like a cough, but shriller, almost resembling a howl. It was repeated every five or six seconds during the whole of the waking moments, and subsided during sleep. Under rest and free purgation the patient recovered, but the paroxysms continued during prolonged intervals, and in the last six years they only lasted from twenty-four to forty-eight hours.
Parker reports four rebellious cases of singultus successfully treated by dry cups applied to the abdomen. In each case it was necessary to repeat the operation after two hours, but recovery was then rapid. Tatevosoff reports a brilliant cure in a patient with chronic chest trouble, by the use of common snuff, enough being given several times to induce lively sneezing. Griswold records a successful treatment of one case in a man of fifty, occurring after a debauch, by the administration of glonoin, 1/150 of a grain every three hours. Heidenhain records a very severe and prolonged case caused, as shown later at the operation and postmortem examination, by carcinoma of the pancreas. The spasms were greatly relieved by cocain administered by the mouth, as much as 15 grains being given in twelve hours. Laborde and Lepine report the case of a young girl who was relieved of an obstinate case of hiccough lasting four days by traction on her tongue. After the tongue had been held out of the mouth for a few minutes the hiccoughs ceased. Laborde referred to two cases of a similar character reported by Viand.
Anomalous Sneezing.—In the olden times sneezing was considered a good omen, and was regarded as a sacred sign by nearly all of the ancient peoples. This feeling of reverence was already ancient in the days of Homer. Aristotle inquired into the nature and origin of the superstition, somewhat profanely wondering why sneezing had been deified rather than coughing. The Greeks traced the origin of the sacred regard for sneezing to the days of Prometheus, who blessed his man of clay when he sneezed. According to Seguin the rabbinical account says that only through Jacob's struggle with the angel did sneezing cease to be an act fatal to man. Not only in Greece and Rome was sneezing revered, but also by races in Asia and Africa, and even by the Mexicans of remote times. Xenophon speaks of the reverence as to sneezing, in the court of the King of Persia. In Mesopotamia and some of the African towns the populace rejoiced when the monarch sneezed. In the present day we frequently hear "God bless you" addressed to persons who have just sneezed, a perpetuation of a custom quite universal in the time of Gregory the Great, in whose time, at a certain season, the air was filled with an unwholesome vapor or malaria which so affected the people that those who sneezed were at once stricken with death-agonies. In this strait the pontiff is said to have devised a form of prayer to be uttered when the paroxysm was seen to be coming on, and which, it was hoped, would avert the stroke of the death-angel.
There are some curious cases of anomalous sneezing on record, some of which are possibly due to affections akin to our present "hay fever," while others are due to causes beyond our comprehension. The Ephemerides records a paroxysm of continual sneezing lasting thirty days. Bonet, Lancisi, Fabricius Hildanus, and other older observers speak of sneezing to death. Morgagni mentions death from congestion of the vasa cerebri caused by sneezing. The Ephemerides records an instance of prolonged sneezing which was distinctly hereditary.
Ellison makes an inquiry for treatment of a case of sneezing in a white child of ten. The sneezing started without apparent cause and would continue 20 or 30 times, or until the child was exhausted, and then stop for a half or one minute, only to relapse again. Beilby speaks of a boy of thirteen who suffered constant sneezing (from one to six times a minute) for one month. Only during sleep was there any relief. The patient recovered under treatment consisting of active leeching, purgation, and blisters applied behind the ear, together with the application of olive oil to the nostrils.
Lee reports a remarkable case of yawning followed by sneezing in a girl of fifteen who, just before, had a tooth removed without difficulty. Half an hour afterward yawning began and continued for five weeks continuously. There was no pain, no illness, and she seemed amused at her condition. There was no derangement of the sexual or other organs and no account of an hysteric spasm. Potassium bromid and belladonna were administered for a few days with negative results, when the attacks of yawning suddenly turned to sneezing. One paroxysm followed another with scarcely an interval for speech. She was chloroformed once and the sneezing ceased, but was more violent on recovery therefrom. Ammonium bromid in half-drachm doses, with rest in bed for psychologic reasons, checked the sneezing. Woakes presented a paper on what he designated "ear-sneezing," due to the caking of cerumen in one ear. Irritation of the auricular branch of the vagus was produced, whence an impression was propagated to the lungs through the pulmonary branches of the vagus. Yawning was caused through implication of the third division of the 5th nerve, sneezing following from reflex implication of the spinal nerves of respiration, the lungs being full of air at the time of yawning. Woakes also speaks of "ear-giddiness" and offers a new associate symptom—superficial congestion of the hands and forearm.
A case of anomalous sneezing immediately prior to sexual intercourse is mentioned on page 511.
Hemophilia is an hereditary, constitutional fault, characterized by a tendency to uncontrollable bleeding, either spontaneous or from slight wounds. It is sometimes associated with a form of arthritis (Ogler). This hemorrhagic diathesis has been known for many years; and the fact that there were some persons who showed a peculiar tendency to bleed after wounds of a trifling nature is recorded in some of the earliest medical literature. Only recently, however, through the writings of Buel, Otto, Hay, Coates, and others, has the hereditary nature of the malady and its curious mode of transmission through the female line been known. As a rule the mother of a hemophile is not a "bleeder" herself, but is the daughter of one. The daughters of a hemophile, though healthy and free from any tendency themselves, are almost certain to transmit the disposition to the male offspring. The condition generally appears after some slight injury in the first two years of life; but must be distinguished from the hemorrhagic affections of the new-born, which will be discussed later. The social condition of the family does not alter the predisposition; the old Duke of Albany was a "bleeder"; and bleeder families are numerous, healthy looking, and have fine, soft skins.
The duration of this tendency, and its perpetuation in a family, is remarkable. The Appleton-Swain family of Reading, Mass., has shown examples for two centuries. Osler has been advised of instances already occurring in the seventh generation. Kolster has investigated hemophilia in women, and reports a case of bleeding in the daughter of a hemophilic woman. He also analyzes 50 genealogic trees of hemophilic families, and remarks that Nasse's law of transmission does not hold true. In 14 cases the transmission was direct from the father to the child, and in 11 cases it was direct from the mother to the infant.
The hemorrhagic symptoms of bleeders may be divided into external bleedings, either spontaneous or traumatic; interstitial bleedings, petechiae, and ecchymoses; and the joint-affections. The external bleedings are seldom spontaneous, and generally follow cuts, bruises, scratches, and often result seriously. A minor operation on a hemophile may end in death; so slight an operation as drawing a tooth has been followed by the most disastrous consequences.
Armstrong, Blagden, and Roberts, have seen fatal hemorrhage after the extraction of teeth. MacCormac observed five bleeders at St. Thomas Hospital, London, and remarks that one of these persons bled twelve days after a tooth-extraction. Buchanan and Clay cite similar instances. Cousins mentions an individual of hemorrhagic diathesis who succumbed to extensive extravasation of blood at the base of the brain, following a slight fall during an epileptic convulsion. Dunlape reports a case of hemorrhagic diathesis, following suppression of the catamenia, attended by vicarious hemorrhage from the gums, which terminated fatally. Erichsenf cites an instance of extravasation of blood into the calf of the leg of an individual of hemophilic tendencies. A cavity was opened, which extended from above the knee to the heel; the clots were removed, and cautery applied to check the bleeding. There was extension of the blood-cavity to the thigh, with edema and incipient gangrene, necessitating amputation of the thigh, with a fatal termination.
Mackenzie reports an instance of hemophilic purpura of the retina, followed by death. Harkin gives an account of vicarious bleeding from the under lip in a woman of thirty-eight. The hemorrhage occurred at every meal and lasted ten minutes. There is no evidence that the woman was of hemophilic descent.
Of 334 cases of bleeding in hemophilia collected by Grandidier, 169 were from the nose, 43 from the mouth, 15 from the stomach, 36 from the bowels, 16 from the urethra, 17 from the lungs, and a few from the skin of the head, eyelids, scrotum, navel, tongue, finger-tips, vulva, and external ear. Osler remarks that Professor Agnew knew of a case of a bleeder who had always bled from cuts and bruises above the neck, never from those below. The joint-affections closely resemble acute rheumatism. Bleeders do not necessarily die of their early bleedings, some living to old age. Oliver Appleton, the first reported American bleeder, died at an advanced age, owing to hemorrhage from a bed-sore and from the urethra. Fortunately the functions of menstruation and parturition are not seriously interfered with in hemophilia. Menstruation is never so excessive as to be fatal. Grandidier states that of 152 boy subjects 81 died before the termination of the seventh year. Hemophilia is rarely fatal in the first year.
Of the hemorrhagic diseases of the new-born three are worthy of note. In syphilis haemorrhagica neonatorum the child may be born healthy, or just after birth there may appear extensive cutaneous extravasations with bleeding from the mucous surfaces and from the navel; the child may become deeply jaundiced. Postmortem examination shows extensive extravasations into the internal viscera, and also organic syphilitic lesions.
Winckel's disease, or epidemic hemoglobinuria, is a very fatal affection, sometimes epidemic in lying-in institutions; it develops about the fourth day after birth. The principal symptom is hematogenous icterus with cyanosis,—the urine contains blood and blood-coloring matter. Some cases have shown in a marked degree acute fatty degeneration of the internal organs—Buhl's disease.
Apart from the common visceral hemorrhages, the results of injuries at birth, bleeding from one or more of the surfaces is a not uncommon event in the new-born, particularly in hospital-practice. According to Osler Townsend reports 45 cases in 6700 deliveries, the hemorrhage being both general and from the navel alone. Bleeding also occurs from the bowels, stomach, and mouth, generally beginning in the first week, but in rare instances it is delayed to the second or third. Out of 50 cases collected by Townsend 31 died and 19 recovered. The nature of the disease is unknown, and postmortem examination reveals no pathologic changes, although the general and not local nature of the affection, its self-limited character, the presence of fever, and the greater prevalence of the disease in hospitals, suggest an infectious origin (Townsend). Kent a speaks of a new-born infant dying of spontaneous hemorrhage from about the hips.
Infantile scurvy, or Barlow's disease, has lately attracted marked attention, and is interesting for the numerous extravasations and spontaneous hemorrhages which are associated with it. A most interesting collection of specimens taken from the victims of Barlow's disease were shown in London in 1895.
In an article on the successful preventive treatment of tetanus neonatorum, or the "scourge of St. Kilda," of the new-born, Turner says the first mention of trismus nascentium or tetanus neonatorum was made by Rev. Kenneth Macaulay in 1764, after a visit to the island of St. Kilda in 1758. This gentleman states that the infants of this island give up nursing on the fourth or fifth day after birth; on the seventh day their gums are so clinched together that it is impossible to get anything down their throats; soon after this they are seized with convulsive fits and die on the eighth day. So general was this trouble on the island of St. Kilda that the mothers never thought of making any preparation for the coming baby, and it was wrapped in a dirty piece of blanket till the ninth or tenth day, when, if the child survived, the affection of the mother asserted itself. This lax method of caring for the infant, the neglect to dress the cord, and the unsanitary condition of the dwellings, make it extremely probable that the infection was through the umbilical cord. All cases in which treatment was properly carried out by competent nurses have survived. This treatment consisted in dressing the cord with iodoform powder and antiseptic wool, the breast-feeding of the baby from the first, and the administration of one-grain doses of potassium bromid at short intervals. The infant death-rate on the island of St. Kilda has, consequently, been much reduced. The author suggests the use of a new iodin-preparation called loretin for dressing the cord. The powder is free from odor and is nonpoisonous.
Human Parasites.—Worms in the human body are of interest on account of the immense length some species attain, the anomalous symptoms which they cause, or because of their anomalous location and issue. According to modern writers the famous Viennese collection of helminths contains chains of tenia saginata 24 feet long. The older reports, according to which the taenia solium (i.e., generally the taenia saginata) grew to such lengths as 40, 50, 60, and even as much as 800 yards, are generally regarded as erroneous. The observers have apparently taken the total of all the fragments of the worm or worms evacuated at any time and added them, thus obtaining results so colossal that it would be impossible for such an immense mass to be contained in any human intestine.
The name solium has no relation to the Latin solus, or solium. It is quite possible for a number of tapeworms to exist simultaneously in the human body. Palm mentions the fact of four tapeworms existing in one person; and Mongeal has made observations of a number of cases in which several teniae existed simultaneously in the stomach. David speaks of the expulsion of five teniae by the ingestion of a quantity of sweet wine. Cobbold reports the case of four simultaneous tapeworms; and Aguiel describes the case of a man of twenty-four who expelled a mass weighing a kilogram, 34.5 meters long, consisting of several different worms. Garfinkel mentions a case which has been extensively quoted, of a peasant who voided 238 feet of tapeworms, 12 heads being found. Laveran reports a case in which 23 teniae were expelled in the same day. Greenhow mentions the occurrence of two teniae mediocanellata.
The size of a tapeworm in a small child is sometimes quite surprising. Even the new-born have exhibited signs of teniae, and Haussmann has discussed this subject. Armor speaks of a fully-matured tapeworm being expelled from a child five days old. Kennedy reports cases in which tapeworms have been expelled from infants five, and five and one-half months old. Heisberg gives an account of a tapeworm eight feet in length which came from a child of two. Twiggs describes a case in which a tapeworm 36 feet long was expelled from a child of four; and Fabre mentions the expulsion of eight teniae from a child. Occasionally the tapeworm is expelled from the mouth. Such cases are mentioned by Hitch and Martel. White speaks of a tapeworm which was discharged from the stomach after the use of an emetic. Lile mentions the removal of a tapeworm which had been in the bowel twenty-four years.
The peculiar effects of a tapeworm are exaggerated appetite and thirst, nausea, headaches, vertigo, ocular symptoms, cardiac palpitation, and Mursinna has even observed a case of trismus, or lockjaw, due to taenia solium. Fereol speaks of a case of vertigo, accompanied with epileptic convulsions, which was caused by teniae. On the administration of kousso three heads were expelled simultaneously. There is a record of an instance of cardiac pulsation rising to 240 per minute, which ceased upon the expulsion of a large tapeworm. It is quite possible for the presence of a tapeworm to indirectly produce death. Garroway describes a case in which death was apparently imminent from the presence of a tapeworm. Kisel has recorded a fatal case of anemia, in a child of six, dependent on teniae.
The number of ascarides or round-worms in one subject is sometimes enormous. Victor speaks of 129 round-worms being discharged from a child in the short space of five days. Pole mentions the expulsion of 441 lumbricoid worms in thirty-four days, and Fauconneau-Dufresne has reported a most remarkable case in which 5000 ascarides were discharged in less than three years, mostly by vomiting. The patient made an ultimate recovery.
There are many instances in which the lumbricoid worms have pierced the intestinal tract and made their way to other viscera, sometimes leading to an anomalous exit. There are several cases on record in which the lumbricoid worms have been found in the bladder. Pare speaks of a case of this kind during a long illness; and there is mention of a man who voided a worm half a yard long from his bladder after suppression of urine. The Ephemerides contains a curious case in which a stone was formed in the bladder, having for its nucleus a worm. Fontanelle presented to the Royal Academy of Medicine of Paris several yards of tapeworm passed from the urethra of a man of fifty-three. The following is a quotation from the British Medical Journal: "I have at present a patient passing in his urine a worm-like body, not unlike a tapeworm as far as the segments and general appearance are concerned, the length of each segment being about 1/4 inch, the breadth rather less; sometimes 1 1/2 segments are joined together. The worm is serrated on the one side, each segment having 1 1/2 cusps. The urine pale, faintly acid at first, within the last week became almost neutral. There was considerable vesical irritation for the first week, with abundant mucus in the urine, specific gravity was 1010; there were no albumin nor tube-casts nor uric acid in the urinary sediments. Later there were pus-cells and abundant pus. Tenderness existed behind the prostate and along the course of left ureter. Temperature of patient oscillated from 97.5 degrees to 103.2 degrees F. There was no history at any time of recto-vesical fistula. Can anyone suggest the name, etc., of this helminth?"
Other cases of worms in the bladder are mentioned in Chapter XIII
Mitra speaks of the passage of round-worms through the umbilicus of an adult; and there is a case mentioned in which round-worms about seven inches long were voided from the navel of a young child. Borgeois speaks of a lumbricoid worm found in the biliary passages, and another in the air passages.
Turnbull has recorded two cases of perforation of the tympanic membrane from lumbricoides. Dagan speaks of the issue of a lumbricoid from the external auditory meatus. Laughton reports an instance of lumbricoid in the nose. Rake speaks of asphyxia from a round-worm. Morland mentions the ejection of numerous lumbricoid worms from the mouth.
Worms have been found in the heart; and it is quite possible that in cases of trichinosis, specimens of the trichinae may be discovered anywhere in the line of cardiac or lymphatic circulation. Quoted by Fournier, Lapeyronnie has seen worms in the pericardial sac, and also in the ventricle. There is an old record of a person dying of intestinal worms, one of which was found in the left ventricle. Castro and Vidal speak of worms in the aorta. Rake reports a case of sudden death from round-worm; and Brown has noted a similar instance.
The echinococcus is a tiny cestode which is the factor in the production of the well-known hydatid cysts which may be found in any part of the body. Delafield and Prudden report the only instance of multilocular echinococcus seen in this country. Their patient was a German who had been in this country five years. There are only about 100 of these cases on record, most of them being in Bavaria and Switzerland.
The filaria sanguinis hominis is a small worm of the nematode species, the adult form of which lives in the lymphatics, and either the adult or the prematurely discharged ova (Manson) block the lymph-channels, producing the conditions of hematochyluria, elephantiasis, and lymph-scrotum. The Dracunculus medinensis or Guinea-worm is a widely-spread parasite in parts of Africa and the West Indies. According to Osler several cases have occurred in the United States. Jarvis reports a case in a post-chaplain who had lived at Fortress Monroe, Va., for thirty years. Van Harlingen's patient, a man of forty-seven, had never lived out of Philadelphia, so that the worm must be included among the parasites infesting this country.
In February, 1896, Henry of Philadelphia showed microscopic slides containing blood which was infested with numbers of living and active filaria embryos. The blood was taken from a colored woman at the Woman's Hospital, who developed hematochyluria after labor. Henry believed that the woman had contracted the disease during her residence in the Southern States.
Curran gives quite an exhaustive article on the disease called in olden times "eaten of worms,"—a most loathsome malady. Herod the Great, the Emperor Galerius, and Philip II of Spain perished from it. In speaking of the Emperor Galerius, Dean Milman, in his "History of Latin Christianity," says, "a deep and fetid ulcer preyed on the lower parts of his body and ate them away into a mass of living corruption." Gibbon, in his "Decline and Fall," also says that "his (Galerius's) death was caused by a very painful and lingering disorder. His body, swelled by an intemperate course of life to an unwieldy corpulence, was covered with ulcers and devoured by immense swarms of those insects who have given their names to this most loathsome disease." It is also said that the African Vandal King, the Arian Huneric, died of the disease. Antiochus, surnamed the "Madman," was also afflicted with it; and Josephus makes mention of it as afflicting the body of Herod the Great. The so-called "King Pym" died of this "morbus pedicularis," but as prejudice and passion militated against him during his life and after his death, this fact is probably more rumor than verity. A case is spoken of by Curran, which was seen by an army-surgeon in a very aged woman in the remote parts of Ireland, and another in a female in a dissecting-room in Dublin. The tissues were permeated with lice which emerged through rents and fissures in the body.
Instances of the larvae of the estrus or the bot-fly in the skin are not uncommon. In this country Allen removed such larvae from the skin of the neck, head, and arm of a boy of twelve. Bethune, Delavigne, Howship, Jacobs, Jannuzzi and others, report similar cases. These flesh-flies are called creophilae, and the condition they produce is called myiosis. According to Osler, in parts of Central America, the eggs of a bot-fly, called the dermatobia, are not infrequently deposited in the skin, and produce a swelling very like the ordinary boil. Matas has described a case in which the estrus larvae were found in the gluteal region. Finlayson of Glasgow has recently reported an interesting case in a physician who, after protracted constipation and pain in the back and sides, passed large numbers of the larvae of the flower-fly, anthomyia canicularis, and there are other instances of myiosis interna from swallowing the larvae of the common house-fly.
There are forms of nasal disorder caused by larvae, which some native surgeons in India regard as a chronic and malignant ulceration of the mucous membranes of the nose and adjacent sinuses in the debilitated and the scrofulous. Worms lodging in the cribriform plate of the ethmoid feed on the soft tissues of that region. Eventually their ravages destroy the olfactory nerves, with subsequent loss of the sense of smell, and they finally eat away the bridge of the nose. The head of the victim droops, and he complains of crawling of worms in the interior of the nose. The eyelids swell so that the patient cannot see, and a deformity arises which exceeds that produced by syphilis. Lyons says that it is one of the most loathsome diseases that comes under the observation of medical men. He describes the disease as "essentially a scrofulous inflammation of the Schneiderian membrane, ... which finally attacks the bones." Flies deposit their ova in the nasal discharges, and from their infection maggots eventually arise. In Sanskrit peenash signifies disease of the nose, and is the Indian term for the disease caused by the deposition of larvae in the nose. It is supposed to be more common in South America than in India.
CHAPTER XVI.
ANOMALOUS SKIN-DISEASES.
Ichthyosis is a disease of the skin characterized by a morbid development of the papillae and thickening of the epidermic lamellae; according as the skin is affected over a larger or smaller area, or only the epithelial lining of the follicles, it is known as ichthyosis diffusa, or ichthyosis follicularis. The hardened masses of epithelium develop in excess, the epidermal layer loses in integrity, and the surface becomes scaled like that of a fish. Ichthyosis may be congenital, and over sixty years ago Steinhausen described a fetal monster in the anatomic collection in Berlin, the whole surface of whose body was covered with a thick layer of epidermis, the skin being so thick as to form a covering like a coat-of-mail. According to Rayer the celebrated "porcupine-man" who exhibited himself in England in 1710 was an example of a rare form of ichthyosis. This man's body, except the face, the palms of the hands, and the soles of the feet, was covered with small excrescences in the form of prickles. These appendages were of a reddish-brown color, and so hard and elastic that they rustled and made a noise when the hand was passed over their surfaces. They appeared two months after birth and fell off every winter, to reappear each summer. In other respects the man was in very good health. He had six children, all of whom were covered with excrescences like himself. The hands of one of these children has been represented by Edwards in his "Gleanings of Natural History." A picture of the hand of the father is shown in the fifty-ninth volume of the Philosophical Transactions.
Pettigrew mentions a man with warty elongations encasing his whole body. At the parts where friction occurred the points of the elongations were worn off. This man was called "the biped armadillo." His great grandfather was found by a whaler in a wild state in Davis's Straits, and for four generations the male members of the family had been so encased. The females had normal skins. All the members of the well-known family of Lambert had the body covered with spines. Two members, brothers, aged twenty-two and fourteen, were examined by Geoffroy-Saint-Hilaire. This thickening of the epidermis and hair was the effect of some morbid predisposition which was transmitted from father to son, the daughters not being affected. Five generations could be reckoned which had been affected in the manner described.
The "porcupine-man" seen by Baker contracted small-pox, and his skin was temporarily freed from the squamae, but these reappeared shortly afterward. There are several older records of prickly men or porcupine-men. Ascanius mentions a porcupine-man, as do Buffon and Schreber. Autenreith speaks of a porcupine-man who was covered with innumerable verrucae. Martin described a remarkable variety of ichthyosis in which the skin was covered with strong hairs like the bristles of a boar. When numerous and thick the scales sometimes assumed a greenish-black hue. An example of this condition was the individual who exhibited under the name of the "alligator-boy." Figure 286 represents an "alligator-boy" exhibited by C. T. Taylor. The skin affected in this case resembled in color and consistency that of a young alligator. It was remarked that his olfactory sense was intact.
The harlequin fetus, of which there are specimens in Guy's Hospital, London Hospital, and the Royal College of Surgeons Museum, is the result of ichthyosis congenita. According to Crocker either after the removal of the vernix caseosa, which may be thick, or as the skin dries it is noticeably red, smooth, shiny, and in the more severe cases covered with actual plates. In the harlequin fetus the whole surface of the body is thickly covered with fatty epidermic plates, about 1/16 inch in thickness, which are broken up by horizontal and vertical fissures, and arranged transversely to the surface of the body like a loosely-built stone wall. After birth these fissures may extend down into the corium, and on movement produce much pain. The skin is so stiff and contracted that the eyes cannot be completely opened or shut, the lips are too stiff to permit of sucking, and are often inverted; the nose and ears are atrophied, the toes are contracted and cramped, and, if not born dead, the child soon dies from starvation and loss of heat. When the disease is less severe the child may survive some time. Crocker had a patient, a male child one month old, who survived three months. Hallopeau and Elliot also report similar cases.
Contagious follicular keratosis is an extremely rare affection in which there are peculiar, spine-like outgrowths, consisting in exudations of the mouths of the sebaceous glands. Leloir and Vidal shorten the name to acne cornee.
Erasmus Wilson speaks of it as ichthyosis sebacea cornea. H. G. Brooke describes a case in a girl of six. The first sign had been an eruption of little black spots on the nape of the neck. These spots gradually developed into papules, and the whole skin took on a dirty yellow color. Soon afterward the same appearances occurred on both shoulders, and, in the same order, spread gradually down the outer sides of the arms—first black specks, then papules, and lastly pigmentation. The black specks soon began to project, and comedo-like plugs and small, spine-like growths were produced. Both the spines and plugs were very hard and firmly-rooted. They resisted firm pressure with the forceps, and when placed on sheets of paper rattled like scraps of metal. A direct history of contagion was traced from this case to others.
Mibelli describes an uncommon form of keratodermia (porokeratosis). The patient was a man of twenty-one, and exhibited the following changes in his skin: On the left side of the neck, beyond a few centimeters below the lobe of the ear, there were about ten small warty patches, irregularly scattered, yellowish-brown in color, irregular in outline, and varying in size from a lentil to a half-franc piece, or rather more. Similar patches were seen on other portions of the face. Patches of varying size and form, sharply limited by a kind of small, peripheral "dike," sinuous but uninterrupted, of a color varying from red to whitish-red, dirty white, and to a hue but little different from that of the healthy skin. Similar patches were seen on the right hand, and again on the back of the right hand was a wide space, prolonged upward in the form of a broad band on the posterior surface of the forearm to just below the olecranon, where the skin was a little smoother and thinner than the surrounding skin, and altogether bare of hairs. The disease was noticed at the age of two, and gradually progressed. The patient always enjoyed the most perfect health, but had contracted syphilis three years before. A brother of the patient, aged twenty-four, for sixteen years has had the same skin-affection as this patient, on the back of the hand, and the sister and father had noticed similar lesions.
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 breasts; 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 sebum 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 circumscribed 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 analogous 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 buttocks, and the thighs, down to and involving the popliteal spaces. The edges of the indurated skin were sharply defined, irregular, and map-like. The affected skin was stretched, but not shiny, 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 buttocks 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 substituted, 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 circumstance 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 friendship and society, and sometimes interfered with his travels. On one occasion a steamboat captain refused to take him as a passenger. 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 masses developed on the skin of the back, buttock, and occiput. In the right pectoral and posterior aspect of the right axillary region, and over the buttocks, 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 assistance 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 Washington.
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 class 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 Washington, 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 horny 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 horny change.
The disease is not usually symmetric, as formerly stated, nor is it simultaneous in different toes. There are no associated constitutional 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 horny. 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 brass 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 passed around the middle phalanx. The nails on the absorbed phalanges had become small and considerably thickened plates. No analogous 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 associate 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, constitutional 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 illustrations. 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 circumstances, 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 dermatitis 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, anus, 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 dermatitis has been given to an epidemic skin-disease which made its appearance in 1891 in England; 425 cases were collected in six institutions, 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 illustrate 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 analogous 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, buttocks, 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 classified 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 illustration. The patient was a girl of eighteen whose face, left breast, anus, 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, associated 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 pricked 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 constitution. 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, passing 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 constitutional 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 button-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 spaces, 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 sexes, 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 button, 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.
Pigmentary Processes.—Friction, pressure, or scratching, if long continued, may produce extensive and permanent pigmentation. This is seen in its highest degree in itching diseases like prurigo and pityriasis. Greenhow has published instances of this kind under the name of "vagabond's disease," a disease simulating morbus addisonii, and particularly found in tramps and vagrants. In aged people this condition is the pityriasis nigra of Willan. According to Crocker in two cases reported by Thibierge, the oral mucous membrane was also stained. Carrington and Crocker both record cases of permanent pigmentation following exposure to great cold. Gautier is accredited with recording in 1890 the case of a boy of six in whom pigmented patches from sepia to almost black began to form at the age of two, and were distributed all over the body. Precocious maturity of the genital organs preceded and accompanied the pigmentation, but the hair was illy developed.
Chloasma uterinum presents some interesting anomalies. Swayne records a singular variety in a woman in whom, during the last three months of three successive pregnancies, the face, arms, hands, and legs were spotted like a leopard, and remained so until after her confinement. Crocker speaks of a lady of thirty whose skin during each pregnancy became at first bronze, as if it had been exposed to a tropical sun, and then in spots almost black. Kaposi knew a woman with a pigmented mole two inches square on the side of the neck, which became quite black at each pregnancy, and which was the first recognizable sign of her condition. It is quite possible that the black disease of the Garo Hills in Assam is due to extreme and acute development of a pernicious form of malaria. In chronic malaria the skin may be yellowish, from a chestnut-brown to a black color, after long exposure to the influence of the fever. Various fungi, such as tinea versicolor and the Mexican "Caraati," may produce discoloration on the skin.
Acanthosis Nigricans may be defined as a general pigmentation with papillary mole-like growths. In the "International Atlas of Rare Skin Diseases" there are two cases pictured, one by Politzer in a woman of sixty-two, and the other by Janovsky in a man of forty-two. The regions affected were mostly of a dirty-brown color, but in patches of a bluish-gray. The disease began suddenly in the woman, but gradually in the man. Crocker has reported a case somewhat similar to these two, under the head of general bronzing without constitutional symptoms, in a Swedish sailor of twenty-two, with rapid onset of pigmentation.
Xeroderma pigmentosum, first described by Kaposi in 1870, is a very rare disease, but owing to its striking peculiarities is easily recognized. Crocker saw the first three cases in England, and describes one as a type. The patient was a girl of twelve, whose general health and nutrition were good. The disease began when she was between twelve and eighteen months old, without any premonitory symptom. The disease occupied the parts habitually uncovered in childhood. The whole of these areas was more or less densely speckled with pigmented, freckle-like spots, varying in tint from a light, raw umber to a deep sepia, and in size from a pin's head to a bean, and of a roundish and irregular shape. Interspersed among the pigment-spots, but not so numerous, were white atrophic spots, which in some parts coalesced, forming white, shining, cicatrix-like areas. The skin upon this was finely wrinkled, and either smooth or shiny, or covered with thin, white scales. On these white areas bright red spots were conspicuous, due to telangiectasis, and there were also some stellate vascular spots and strife interspersed among the pigment. Small warts were seen springing up from some of the pigment spots. These warts ulcerated and gave rise to numerous superficial ulcerations, covered with yellow crusts, irregularly scattered over the face, mostly on the right side. The pus coming from these ulcers was apparently innocuous. The patient complained neither of itching nor of pain. Archambault has collected 60 cases, and gives a good resume to date. Amiscis reports two cases of brothers, in one of whom the disease began at eight months, and in the other at a year, and concludes that it is not a lesion due to external stimuli or known parasitic elements, but must be regarded as a specific, congenital dystrophy of the skin, of unknown pathogenesis. However, observations have shown that it may occur at forty-three years (Riehl), and sixty-four years (Kaposi). Crocker believes that the disease is an atrophic degeneration of the skin, dependent on a primary neurosis, to which there is a congenital predisposition.
Nigrities is a name given by the older writers to certain black blotches occurring on the skin of a white person—in other words, it is a synonym of melasma. According to Rayer it is not uncommon to see the scrotum and the skin of the penis of adults almost black, so as to form a marked contrast with the pubes and the upper part of the thighs. Haller met with a woman in whom the skin of the pubic region was as black as that of a negress. During nursing the nipples assume a deep black color which disappears after weaning. Le Cat speaks of a woman of thirty years, whose forehead assumed a dusky hue of the color of iron rust when she was pregnant about the seventh month. By degrees the whole face became black except the eyes and the edges of the lips, which retained their natural color. On some days this hue was deeper than on others; the woman being naturally of a very fair complexion had the appearance of an alabaster figure with a black marble head. Her hair, which was naturally exceedingly dark, appeared coarser and blacker. She did not suffer from headache, and her appetite was good. After becoming black, the face was very tender to the touch. The black color disappeared two days after her accouchement, and following a profuse perspiration by which the sheets were stained black. Her child was of a natural color. In the following pregnancy, and even in the third, the same phenomenon reappeared in the course of the seventh month; in the eighth month it disappeared, but in the ninth month this woman became the subject of convulsions, of which she had one each day. The existence of accidental nigrities rests on well-established facts which are distinctly different from the pigmentation of purpura, icterus, or that produced by metallic salts. Chomel quotes the case of a very apathic old soldier, whose skin, without any appreciable cause, became as brown as that of a negro in some parts, and a yellowish-brown in others. Rustin has published the case of a woman of seventy who became as black as a negress in a single night. Goodwin relates the case of an old maiden lady whose complexion up to the age of twenty-one was of ordinary whiteness, but then became as black as that of an African. Wells and Rayer have also published accounts of cases of accidental nigrities. One of the latter cases was a sailor of sixty-three who suffered from general nigrities, and the other was in a woman of thirty, appearing after weaning and amenorrhea.
Mitchell Bruce has described an anomalous discoloration of the skin and mucous membranes resembling that produced by silver or cyanosis. The patient, a harness-maker of forty-seven, was affected generally over the body, but particularly in the face, hands, and feet. The conjunctival, nasal, and aural mucosa were all involved. The skin felt warm, and pressure did not influence the discoloration. The pains complained of were of an intermittent, burning, shooting character, chiefly in the epigastric and left lumbar regions. The general health was good, and motion and sensation were normal. Nothing abnormal was discovered in connection with the abdominal and thoracic examinations. The pains and discoloration had commenced two years before his admission, since which time the skin had been deepening in tint. He remained under observation for three months without obvious change in his symptoms. There was nothing in the patient's occupation to account for the discoloration. A year and a half previously he had taken medicine for his pains, but its nature could not be discovered. He had had syphilis.
Galtier mentions congenital and bronze spots of the skin. A man born in Switzerland the latter part of the last century, calling himself Joseph Galart, attracted the attention of the curious by exhibiting himself under the name of the "Living Angel." He presented the following appearance: The skin of the whole posterior part of the trunk, from the nape of the neck to the loins, was of a bronze color. This color extended over the shoulders and the sides of the neck, and this part was covered with hairs of great fineness and growing very thick; the skin of the rest of the body was of the usual whiteness. Those parts were the darkest which were the most covered with hair; on the back there was a space of an inch in diameter, which had preserved its whiteness, and where the hairs were fewer in number, darker at their bases, and surrounded by a very small black circle; the hair was thinner at the sides of the neck; there were a great many individual hairs surrounded by circles of coloring matter; but there were also many which presented nothing of this colored areola. In some places the general dark color of the skin blended with the areola surrounding the roots of the hair, so that one uniform black surface resulted. In many places the dark color changed into black. The irides were brown. The man was of very unstable character, extremely undecided in all his undertakings, and had a lively but silly expression of countenance. A distinct smell, as of mice, with a mixture of a garlicky odor, was emitted from those parts where the excessive secretion of the coloring matter took place. In those places the heat was also greater than natural. Rayer recites the case of a young man whom he saw, whose eyelids and adjacent parts of the cheeks were of a bluish tint, similar to that which is produced on the skin by the explosion of gunpowder. |
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