Chapter XVI: Anomalous Skin-Diseases (2)

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.

Billard has published an extraordinary case of blue discoloration of the skin in a young laundress of sixteen. Her neck, face, and upper part of the chest showed a beautiful blue tint, principally spreading over the forehead, the alae, and the mouth. When these parts were rubbed with a white towel the blue parts of the skin were detached on the towel, coloring it, and leaving the skin white. The girl's lips were red, the pulse was regular and natural, and her strength and appetite like that of a person in health. The only morbid symptom was a dry cough, but without mucous rattle or any deficiency of the sound of the chest or alteration of the natural beat of the heart. The catamenia had never failed. She had been engaged as a laundress for the past two years. From the time she began this occupation she perceived a blueness around her eyes, which disappeared however on going into the air. The phenomenon reappeared more particularly when irons were heated by a bright charcoal fire, or when she worked in a hot and confined place. The blueness spread, and her breast and abdomen became shaded with an azure blue, which appeared deeper or paler as the circulation was accelerated or retarded. When the patient's face should have blushed, the face became blue instead of red. The changes exhibited were like the sudden transition of shades presented by the chameleon. The posterior part of the trunk, the axillae, the sclerotic coats of the eyes, the nails, and the skin of the head remained in their natural state and preserved their natural color. The linen of the patient was stained blue. Chemical analysis seemed to throw no light on this case, and the patient improved on alkaline treatment. She vomited blood, which contained sufficient of the blue matter to stain the sides of the vessel. She also stated that in hemorrhage from the nose she had seen blue drops among the drops of blood. One cannot but suspect indigo as a factor in the causation of this anomalous coloration.

Artificial discolorations of the skin are generally produced by tattooing, by silver nitrate, mercury, bismuth, or some other metallic salt.

Melasma has been designated as an accidental and temporary blackish discoloration of the skin. There are several varieties: that called Addison's disease, that due to uterine disease, etc. In this affection the skin assumes a dark and even black hue.

Leukoderma is a pathologic process, the result of which is a deficiency in the normal pigmentation of the skin, and possibly its appendages. Its synonyms are leukopathia, vitiligo, achroma, leukasmus, and chloasma album. In India the disease is called sufaid-korh, meaning white leprosy. It has numerous colloquial appellations, such as chumba or phoolyree (Hindoo), buras (Urdu), cabbore (Singalese), kuttam (Taneil), dhabul (Bengal). It differs from albinism in being an acquired deficiency of pigment, not universal and not affecting the eye. Albinism is congenital, and the hair and eyes are affected as well as the skin.

The disease is of universal distribution, but is naturally more noticeable in the dark-skinned races. It is much more common in this country among the negroes than is generally supposed.

The "leopard-boy of Africa," so extensively advertised by dime museums over the country, was a well-defined case of leukoderma in a young mulatto, a fitting parallel for the case of ichthyosis styled the "alligator-boy."

Figure 293 represents a family of three children, all the subjects of leukoderma. Leukoderma is more common among females. It is rarely seen in children, being particularly a disease of middle age. Bissell reports a case in an Indian ninety years of age, subsequent to an attack of rheumatism thirty years previous. It is of varying duration, nearly every case giving a different length of time. It may be associated with most any disease, and is directly attributable to none. In a number of cases collected rheumatism has been a marked feature. It has been noticed following typhoid fever and pregnancy.

In white persons there are spots or blotches of pale, lustreless appearance either irregular or symmetric, scattered over the body. In the negro and other dark-skinned races a mottled appearance is seen. If the process goes to completion, the whole surface changes to white. The hair, though rarely affected, may present a mottled appearance. There seems to be no constitutional disturbances, no radical change in the skin, no pain--in fact, no disturbance worthy of note. The eye is not affected; but in a negro the sclerotic generally appears muddy.

It appears first in small spots, either on the lips, nose, eyelids, soles, palms, or forehead, and increases peripherally--the several spots fusing together. The skin is peculiarly thin and easily irritated. Exposure to the sun readily blisters it, and after the slightest abrasion it bleeds freely. Several cases have been reported in which the specific gravity of the urine was extremely high, due to an excess of urea. Wood calls attention to the wave-like course of leukoderma, receding on one side, increasing on the other. The fading is gradual, and the margins may be abrupt or diffuse. The mucous membranes are rosy. The functions of the swell-glands are unimpaired.

The theory of the absence of pigment causing a loss of the olfactory sense, spoken of by Wallace, is not borne out by several observations of Wood and others. Wilson says: "Leukasma is a neurosis, the result of weakened innervation of the skin, the cause being commonly referable to the organs of assimilation or reproduction." It is not a dermatitis, as a dermatitis usually causes deposition of pigment. The rays of the sun bronze the skin; mustard, cantharides, and many like irritants cause a dermatitis, which is accompanied by a deposition of pigment. Leukoderma is as common in housemaids as in field-laborers, and is in no way attributable to exposure of sun or wind. True leukodermic patches show no vascular changes, no infiltration, but a partial obliteration of the rete mucosum. It has been ascribed to syphilis; but syphilitic leukoderma is generally the result of cicatrices following syphilitic ulceration.

Many observers have noticed that negroes become several degrees lighter after syphilization; but no definite relation between syphilis and leukoderma has yet been demonstrated in this race. Postmortem examinations of leukodermic persons show no change in the suprarenal capsule, a supposed organ of pigmentation.

Climate has no influence. It is seen in the Indians of the Isthmus of Darien, the Hottentots, and the Icelanders. Why the cells of the rete mucosum should have the function in some races of manufacturing or attracting pigment in excess of those of other races, is in itself a mystery. By his experiments on the pigment-cells of a frog Lister has established the relation existing between these elements and innervation, which formerly had been supposititious.

Doubtless a solution of the central control of pigmentation would confirm the best theory of the cause of leukoderma--i.e., faulty innervation of the skin. At present, whether the fault is in the cell proper, the conducting media, or the central center, we are unable to say. It is certainly not due to any vascular disturbances, as the skin shows no vascular changes.

White spots on the nails are quite common, especially on young people. The mechanic cause is the presence of air between the lamellae of the affected parts, but their origin is unknown. According to Crocker in some cases they can be shown to be a part of trophic changes. Bielschowsky records the case of a man with peripheral neuritis, in whom white spots appeared at the lower part of the finger-nails, grew rapidly, and in three weeks coalesced into a band across each nail a millimeter wide. The toes were not affected. Shoemaker mentions a patient who suffered from relapsing fever and bore an additional band for each relapse. Crocker quotes a case reported by Morison of Baltimore, in which transverse bars of white, alternating with the normal color, appeared without ascertainable cause on the finger-nails of a young lady and remained unchanged.

Giovannini describes a case of canities unguium in a patient of twenty-nine, following an attack of typhoid fever. On examining the hands of this patient the nails showed in their entire extent a white, opaque, almost ivory color. An abnormal quantity of air found in the interior of the nails explains in this particular case their impaired appearance. It is certain that the nails, in order to have admitted such a large quantity of air into their interior must have altered in their intimate structure; and Giovannini suggests that they were subject to an abnormal process of keratinization. Unna describes a similar case, which, however, he calls leukonychia.

Plica polonica, or, as it was known in Cracow--weicselzopf, is a disease peculiar to Poland, or to those of Polish antecedents, characterized by the agglutination, tangling, and anomalous development of the hair, or by an alteration of the nails, which become spongy and blackish. In older days the disease was well known and occupied a prominent place in books on skin-diseases. Hercules de Saxonia and Thomas Minadous, in 1610, speak of plica as a disease already long known. The greater number of writers fix the date of its appearance in Poland at about the year 1285, under the reign of Lezekle-Noir. Lafontaine stated that in the provinces of Cracow and Sandomir plica formerly attacked the peasantry, beggars, and Jews in the proportion of 1 1/2 in 20; and the nobility and burghers in the proportion of two in 30 or 40. In Warsaw and surrounding districts the disease attacked the first classes in the proportion of one to ten, and in the second classes one to 30. In Lithuania the same proportions were observed as in Warsaw; but the disease has gradually grown rarer and rarer to the present day, although occasional cases are seen even in the United States.

Plica has always been more frequent on the banks of the Vistula and Borysthenes, in damp and marshy situations, than in other parts of Poland. The custom formerly prevailing in Poland of shaving the heads of children, neglect of cleanliness, the heat of the head-dress, and the exposure of the skin to cold seem to favor the production of this disease.

Plica began after an attack of acute fever, with pains like those of acute rheumatism in the head and extremities, and possibly vertigo, tinnitus aurium, ophthalmia, or coryza. Sometimes a kind of redness was observed on the thighs, and there was an alteration of the nails, which became black and rough, and again, there was clammy sweat. When the scalp was affected the head was sore to the touch and excessively itchy. A clammy and agglutinating sweat then occurred over the cranium, the hair became unctuous, stuck together, and appeared distended with an adhesive matter of reddish-brown color, believed by many observers to be sanguineous. The hair was so acutely sensitive that the slightest touch occasioned severe pain at the roots. A viscid matter of a very offensive smell, like that of spoiled vinegar, or according to Rayer like that of mice or garlic, exuded from the whole surface of each affected hair. This matter glued the hairs together, at first from their exit at the skin, and then along the entire length; it appeared to be secreted from the whole surface of the scalp and afterward dried into an incrustation. If there was no exudation the disease was called plica sicca. The hair was matted and stuck together in a variety of ways, so as to resemble ropes (plica multiformis). Sometimes these masses united together and formed one single thick club like the tail of a horse (plica caudiformis). Again, and particularly in females, the hair would become matted and glued together into one uniform intricate mass of various magnitudes. The hair of the whole body was likely to be attacked with this disease. Kalschmidt of Jena possessed the pubes of a woman dead of plica, the hair of which was of such length that it must have easily gone around the body. There was formerly a superstition that it was dangerous to cut the hair until the discharge diminished. Lafontaine, Schlegel, and Hartman all assure us that the section of the affected masses before this time has been known to be followed by amaurosis, convulsions, apoplexy, epilepsy, and even death. Alarmed or taught by such occurrences, the common people often went about all their lives with the plica gradually dropping off. Formerly there was much theorizing and discussion regarding the etiology and pathology of plica, but since this mysterious affection has been proved to be nothing more than the product of neglect, and the matting due to the inflammatory exudation, excited by innumerable pediculi, agglutinating the hair together, the term is now scarcely mentioned in dermatologic works. Crocker speaks of a rare form which he entitles neuropathic plica, and cites two cases, one reported by Le Page whose specimen is in the Royal College of Surgeons Museum; and the other was in a Hindoo described by Pestonji. Both occurred in young women, and in both it came on after washing the hair in warm water, one in a few minutes, and the other in a few hours. The hair was drawn up into a hard tangled lump, impossible to unravel, limited to the right side in Le Page's patient, who had very long hair, and in Pestonji's case to the back of the head, where on each side was an elongated mass, very hard and firm, like a rope and about the size of the fist. There was no reason to believe that it was ascribable to imposture; the Hindoo woman cut the lumps off herself and threw them away. Le Page found the most contracted hairs flattened. Stellwagon reports a case of plica in a woman. It occupied a dollar-sized area above the nape of the neck, and in twelve years reached the length of 12 feet. There was no history of its manner of onset.

Tinea nodosa is a name given by Morris and Cheadle to a case of nodular growth on the beard and whiskers of a young man. In a case noticed by Crocker this disease affected the left side of the mustache of a medical man, who complained that the hair, if twisted up, stuck together. When disintegrated the secretion in this case seemed to be composed of fungous spores. Epithelium fragments, probably portions of the internal root-sheath, sometimes adhere to the shaft of the hair as it grows up, and look like concretions. Crocker states that he is informed by White of Boston that this disease is common in America in association with alopecia furfuracea, and is erroneously thought to be the cause of the loss of hair, hence the popular name, "hair-eaters."

Thomson describes a case of mycosis fungoides in a young girl of the age of fourteen, whom he saw in Brussels toward the end of October, 1893. She was the third of a family of 13 children of whom only five survived. Of the children born subsequently to the patient, the first were either premature or died a few days after their births. The seventh was under treatment for interstitial keratitis and tuberculous ulceration of the lips and throat. The disease in the patient made its appearance about seven months previously, as a small raised spot in the middle of the back just above the buttocks. Many of the patches coalesced. At the time of report the lumbar region was the seat of the disease, the affection here presenting a most peculiar appearance, looking as if an enormous butterfly had alighted on the patient's back, with its dark blue wings covered with silvery scales, widely expanded. The patient was not anemic and appeared to be in the best of health. None of the glands were affected. According to Thomson there is little doubt that this disease is caused by non-pyogenic bacteria gaining access to the sweat-glands. The irritation produced by their presence gives rise to proliferation of the connective-tissue corpuscles.

Jamieson reports a case of mycosis in a native of Aberdeenshire aged thirty-eight. There was no history of any previous illness. The disease began three years previous to his application for treatment, as a red, itching, small spot on the cheek. Two years later lumps presented themselves, at first upon his shoulders. The first thing to strike an observer was the offensive odor about the patient. In the hospital wards it made all the occupants sick. The various stages of the disease were marked upon the different parts of the body. On the chest and abdomen it resembled an eczema, on the shoulders there were brown, pinkish-red areas. On the scalp the hair was scanty, the eye-brows denuded, and the eyelashes absent. The forehead was leonine in aspect. From between the various nodosities a continual discharge exuded, the nodosities being markedly irregular over the limbs. The backs of the hands, the dorsums of the feet, the wrists and ankles, had closely approximating growths upon them, while under the thick epidermis of the palms of the hands were blisters. Itching was intense. The patient became emaciated and died thirteen days after his admission into the hospital. A histologic examination showed the sarcomatous nature of the various growths. The disease differed from "button-scurvy." Mycosis fungoides approximates, clinically and histologically, granulomata and sarcomata.

Morris described an interesting case of universal dermatitis, probably a rare variety of mycosis fungoides. The patient had for many years a disease which had first appeared on the arms and legs, and which was usually regarded by the physicians who saw the case as eczema. At times the disease would entirely disappear, but it relapsed, especially during visits to India. At the time the patient came under the care of Morris, his general health seemed unaffected. The skin of the whole body, except the face, the scalp, and the front of the chest, was of a mahogany color. The skin of the lips was so thickened that it could not be pinched into folds, and was of a mottled appearance, due to hemorrhagic spots. All over the thickened and reddened surface were scattered crops of vesicles and boils. The nails were deformed, and the toes beyond the nails were tense with a serous accumulation. The glands in the right axilla and the groin were much enlarged. The hair on the pubes had disappeared. The abdomen was in a condition similar to that upon the limbs, but less in degree. The front of the chest below the nipples was covered with dark papules the size of a pin's head. The back, the buttocks, the face, and the scalp presented similar lesions. The most striking lesions were three ulcers--one on the back of the right hand, one on the right temple, and the other on the left cheek. The largest was the size of a florin, and had elevated borders, somewhat infiltrated; they were covered with a brown, dry scab. The patient suffered from itching at night so that he could not sleep. He was kept under observation, and in spite of treatment the malady advanced in a periodic manner, each exacerbation being preceded by a feeling of tension in the parts, after which a crop of vesicles would appear. Sometimes, especially on the feet, bullae formed. The patient finally left the hospital and died of an intercurrent attack of pneumonia. A microscopic examination revealed a condition which might be found with a number of the chronic affections of the skin, but, in addition, there were certain cell-inclusions which were thought to represent psorosperms. Morris thought this case corresponded more to mycosis fungoides than any other malady.