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.

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,

Date: 2014-12-29; view: 725