20 EAST SIXTY-THIRD Street.

A CASE OF ICHTHYOSIS CONGENITA WITH SOME UNUSUAL FEATURES.1

BY JOHN CABOT, M.D.,

CLINICAL ASSISTANT, VANDERBILT CLINIC, DERMATOLOGICAL DEPARTMENT, NEW YORK.

My aim being to present a practical contribution, I shall omit all general references and discussion, and confine myself to reporting the case, with a brief résumé in conclusion.

Bertha Z-, fourteen months old, born in this country of German parentage, sent to me through the kindness of Dr. Philip Roth, Jr., of Newark, N. J., and first seen November 10, 1894.

The history, as gathered from the mother, is that both parents and two older children, of ten and seven years of age respectively, are healthy. The mother has had no miscarriages or premature births before, but says that this child was born at seven months of utero-gestation, although her abdomen was more distended than with either of her other children at full time. She was four days in labor, with gushes of a watery fluid, accompanied by uterine pain, every halfhour or so, with constant dribbling between pains. The child was weak and marasmic when born. The mucous membrane of the eyelids was much inflamed, ectropion existing. The eyes were open, and there was inability to close them. The nostrils were occluded, and there was difficulty in breathing, so that for some days it was expected to die at any moment.

The skin was thickened, dry, red, and scaly over the entire body at birth, with scales from pin-head size to one and a half inch square, the largest being on the trunk.

Dr. Roth writes, in answer to my inquiry: "In regard to Bertha Z-'s case, would say that there was a great deal of hydramnios, so much so that when I ruptured the bag it seemed to me that it might have been about two gallons. The child was in normal position, L.O.A., and delivered easily. I will state here that I delivered the woman three times, the other children being perfectly healthy. When Bertha was born. she presented the appearance of a Chinese baby, the outer canthi of the eyelids were drawn downward and inward, almost completely closing eyeballs from view.

1 Read before the New York Society of Dermatology and GenitoUrinary Surgery, March 20, 1895.

There was no vernix caseosa on child's head or body, it being perfectly dry when born. The nose was like a white, bony prominence protruding from the face, giving it a most ghastly look; the angles of the mouth were tightly drawn downward and inward, and had a cracked appearance. The ears were bound down to the head and were immovable, and looked as though they were gangrenous. There was no hair on the child's head; both nares were closed, so that I had to make an incision into each one to allow the child to breathe; the rest of the body, that is the skin, had the appearance as though the child was enveloped in oil-silk, shrivelled, and not glistening, but dull."

The baby was nursed at the breast for a short time, but there was so much difficulty from the inability of the child to suck that it was spoon-fed with condensed milk and water.

The marasmus continued for two months, after which time the child gradually gained in weight, and became healthy except for the skin lesion, and occasional attacks of bronchitis. At five months of age it had pertussis, followed by diarrhoea during the hot weather, when it again became marasmic, reviving slowly after a number of weeks' illness, and for the last two months has gained decidedly.

The eyelids were at first held firmly open, with about three-quarters of an inch interval between the upper and lower lids, the conjunctivæ being forced outward, thus diminishing the space of uncovered eyeball to about one-half an inch; this condition gradually improved under simple treatment to the point that the child is now able to bring the lids within half an inch of each other when asleep, the mucous membrane approaching to within one-eighth inch.

The hair was absent at birth, but has grown sparsely, and fallen out twice. The finger-nails have never been entirely normal, growing very long, rupial in character of late, beginning to drop from the fingers about three months ago until there is only one left.

The entire skin has been shed in different portions of the body from three to five times, that on the face and upper part of the body being most frequently thrown off, that of the lower limbs and scalp the least so; the skin peeling off in large and small flakes, leaving a reddened surface almost like natural skin, but which after a few days again becomes dry and wrinkled. In peeling off, islands of the old skin, tense and smooth, were often left, which when extending around the limb, formed tight bands and gave the appearance as if the child was outgrowing its own skin.

There has always been more or less heat in the skin; no moisture or perspiration was ever noticed.

The child has had much and varied treatment by many doctors, but without any effect.

Status Presens.-The child is well nourished, though somewhat smaller than it should be at fourteen months. Weight, 13 lbs. Length of body, 24 inches. Circumference of head, 171⁄2 inches. Chest, 18 inches. Anterior fontanelle still open one-half inch. It is active and intelligent, but makes no effort to crawl or walk; is good-natured, and only restless when the clothing is too warm; takes cold easily. Heart, lungs, liver, and spleen negative; bowels regular. Pulse, 106. Respiration, 38. Urine clear, acid, 1.022; no albumin or sugar. No hair on the head. There is only one fingernail, that on third finger of right hand; toe-nails normal. Ears thickened and lumpy in places; hearing normal. Eyes staring, from inability to close the thickened and stiff lids; ectropion. Sight good. The mouth is held half open, and the tongue is slightly enlarged; there are only two teeth, the lower incisors.

The hands and feet are stiffened, with almost no power of flexion or extension; the child being unable to grasp even large objects with the fingers.

The surface of the body feels warm to the touch, the thermometer showing 101.2° F. in axilla; 100.4° F. in

rectum.

I

The skin over the entire body is slightly reddened, dry, hard, and scaly; scales light gray in color, of papery consistency and from one thirty-second inch to 1 x 2 inches; outlines polygonal, formed by the natural furrows in the skin. The skin is tense in places, notably on the left forearm, right leg, and left thigh and leg, where deep creases are formed by bands of the older skin extending round the limb, above and below which the flesh has seemingly filled out by recent growth, being raised one-quarter inch or more above the constricting bands, giving it a "hide-bound" appearance, which can be seen on the left leg in the photograph. This was taken soon after I first saw the child, and being necessarily an instantaneous exposure, is not as good as could be desired, but it serves to give an idea of the general appearance.

Surrounding the mouth, for an inch or more, the skin is almost natural, thin and white. On the upper part of the forehead is a line of demarcation where the old and new skin are in contact, that on the face being the fourth reproduction, that on the scalp the second or third.

November 28th.-On admission temperature 102° F. (Other physical conditions as noted above.) November 29th.-Temperature, A.M., 100.8° F.; noon, 99.6° F.; P.M., 102° F. 99.6° F.; P.M., 102° F. Constipated. Olive-oil inunctions. Cascara, 3 ss.

November 30th.-Temperature, A.M., 99.8° F.; noon, 100° F.; P.M., 99° F. Stool, yellow and lumpy.

December 1st.-Temperature, A.M., 100° F.; noon, 101° F.; P.M., 103° F. Ice cap. Cloths wet with equal parts linseed and cotton-seed oils to body. Wash of ichthyol and alcohol, aa 3 ij., aqua, 3j., to head, neck, and face. Liquid peptonoids.

December 2d.-Temperature, A.M., 100° F.; noon, 102.4° F.; P.M., 101° F.

December 3d.-Temperature, A.M., 102.4° F.; noon, 102.8° F.; P.M., 101.5° F. Ichthyol wash discontinued. Restless. Phenacetine, gr. j., at 10 P.M.

December 4th.-Temperature, A.M., 103.4° F. Ice cap. Noon, 101° F.; P.M., 103° F. Phenacetine, gr. j., at 9 and 1 P.M.

December 5th.-Temperature, A.M., 104° F. Ice cap. Noon, 101° F.; P.M., 100.8° F.

Treatment. The child was put upon raw linseed oil, one drachm t.i.d., and enveloped in cloths wet with same material. Boric acid solution to eyelids.

November 16, 1894.-Skin about the same condition. Temperature, rectum, 100.2° F.; axilla, 101.2° F.; with surface thermometer on chest, 100.6° F. Pulse, 98. Respiration, 38.

November 23d.-Skin decidedly softer, eyelids come close together. Temperature, rectum, 99.8° F.; axilla, 100.2° F.; on chest, 100° F. Pulse, 96. Respiration, 36.

November 28th.-No change in skin. Temperature, rectum, 101.8° F.; axilla, 102.6° F.; on chest, 102.4° F. Pulse, 110. Respiration, 44. Cough, with fever and restlessness, since last visit. Physical examination shows no dulness on percussion; auscultation is impossible, the crackling of the skin under the ear covering all other sounds. The mother says it always takes more or less cold in journeying back and forth from Newark to New York. On this account, and because it was thought that better facilities for constant observation and more regular applications could be had in a hospital than the mother could give at home, with her hands full of family matters, she was induced to leave the child at the Babies' Hospital, where it came under the care of Drs. L. Emmett Holt and E. B. Bronson. Dr. Holt has obligingly placed the hospital records at my disposal, and from them I make extracts on the course of the disease as follows:

Four or

December 17th.-Temperature, A.M., 103° F.; noon, 102° F.; 7 P.M., 107° F. Ice pack for eight minutes. Temperature, 8 P.M., 98.6° F. Peroxide of hydrogen to suppurating places on head.

Ice

December 18th.-Temperature, 3 A.M., 105° F. pack. Temperature, 3.30 A.M., 98.6° F. At 3 P.M. vomited. Temperature, 100.2° F.

December 19th.-Temperature, 98.6° F.; II A.M., vomited. Noon temperature, 101.8° F.; 9 P.M., 105° F. Ice pack. II P.M., temperature, 97° F. Hot-water bags. Whole back of head is boggy and oedematous ; stools gray.

December 20th.-Temperature, A.M., 100.2° F. Vomited at 8 A.M. Taken home by mother against advice. Skin slightly softer than on entrance.

December 22d.—Died with continuance of fever. In reply to my inquiry as to what was, in his opinion, the direct cause of death, Dr. Holt writes: "The thing which interested me most, while the baby was in the hospital, was the hyperpyrexia, and particularly the wide and rapid fluctuations in the course of the temperature. This is something which I have never seen paralleled in any other infant. It seemed to me to be due to the fact that radiation was very imperfect and at times almost impossible, since with a comparatively small amount of bronchitis the temperature would rise in an hour or two to 108° F., and drop in the course of an hour, under the influence of a bath, nearly to normal.

"I made repeated examinations of the chest while the baby was in the hospital, but never found evidence of anything more than a generalized bronchitis of the large tubes. It was to the general malnutrition rather than to any local disease that the child's decline and final death seemed to me to be due."

Résumé. The etiology in this case would seem to be defective intra-uterine nutrition, resulting in hydramnios, imperfectly developed skin, and premature birth. A curious fact noted by Schroeder is that over seventy-five per cent. of such infants are females. According to modern obstetricians, hydramnios may be due to a morbid condition in either mother or fœtus. There was here no history of syphilis or other disease in father or mother, they always having been in the best of health. Playfair says hydramnios is due to obstructed umbilical circulation, but the condition of placenta and cord was not noted in this case.

No microscopical examination of the skin was made, a small piece taken for this purpose being mislaid after it was in the microscopist's hands, and I was not aware of its loss until it was too late to procure another. Neuman says that the papillæ are enlarged, their bloodvessels dilated, the cutis dense, the lumen of veins narrowed by growth from their interior, the corneous layer thickened, composed of superimposed lamellæ, and the rete between the papillæ much hypertrophied. Hair follicles are absent or lengthened, and contained long hairs, the external root-sheath hypertrophied, the sebaceous glands dilated to a cyst form, the sweat-glands dilated, and the subcutaneous fat diminished. Different observers have noted slightly differing morbid conditions, but agree in the main with the above. Crocker has shown that the horny cells dip down deeper into the regions occupied in the normal skin by the retepegs, and that the adherent and stratified layer of each horny cap is composed at the apex of the papillary growth. The child was born without "vernix caseosa," and there was at no time any hyper-secretion of sebaceous material, so that it would be proper to classify it under the head of "simplex," as distinguished from "sebacea." The skin disease was fairly well-de

veloped at birth, and the thickening increased as time went on, although there was a slight modification of the skin around the mouth and eyes, where it softened to a moderate extent, as shown by the gain in control over the muscles of these regions.

This child lived much longer than is usual in congenital ichthyosis, it being very exceptional to see them live more than a few days when affected to such a marked degree. The well-nourished condition and continuous growth for sixteen months also indicate an unusually good set of digestive apparatus and a large amount of vitality. The constant hyperæmic condition of the skin, the surface temperature on the three occasions when it was taken showing a rise of from two-tenths to six-tenths of a degree over that in the rectum, the axillary temperature rising from fourtenths to one degree over the rectal, is worthy of note, and it would have been interesting if these observations had been continued in the hospital. The rapid desquamation is also somewhat unusual in a simple ichthyosis, where the scales are generally adherent. It seems to me fair to infer that there was also a dermatitis present.

The extreme fluctuations of temperature from seemingly slight causes, as noted by Dr. Holt, are unequal and difficult to account for, except on his theory of imperfect radiation from the surface, though in looking over the history it has some points that make one think of a possible pneumonia or meningitis. Landois says that cutting off the cutaneous respiration in warmblooded animals by coating with an impermeable varnish, or by destruction of skin, as in burns, causes death sooner or later, according to the extent of surface involved, probably from a loss of too much heat. It is accompanied by a fall in bodily temperature and increased frequency in respiration. Neither of these symptoms were present in this case, leaving us to infer that the insensible perspiration was about normal. No autopsy was obtained.

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168 WEST FORTY-EIGHTH STREET.

THE REDUCTION OF MODERATE DEGREES
OF DEFORMITY IN HIP DISEASE.1
BY A. B. JUDSON, M.D.,

NEW YORK.

EXTREME deformity occurring during the progress of hip disease is common only in patients who, for some reason or other, have failed to come under the ordinary methods of treatment. A distressing clinical picture is that of a child lying in bed with the thigh extremely flexed and adducted. But the deformity diminishes. with marvellous rapidity if the weight and pulley, or any of the ordinary forms of traction apparatus is applied. These methods, while they compel a change in the direction of the limb, at the same time protect from inadvertent and painful disturbance of the joint.

The statement may seem unreasonable, but it is nevertheless true, that the more exaggerated the deformity. the more easily it is affected by traction. This is due to the mechanical conditions present. An iron rod

still further straightened, but the straighter it becomes the more difficult it is to make any further advance, and it soon becomes evident that absolute straightness cannot be produced in a rod by traction. Another kind of force may then be employed. Pressure may be made at the convexity of the bent rod and counterpressure at its two ends, as in Fig. 4, when the rod may be easily straightened and even bent the other way. This latter force, pressure and counter-pressure, is applicable and very effective when applied to the knee, represented in Fig. 4, in which case the leverage above and below the joint is sufficient. But there is no such

leverage found in an effort to correct in this way a deformed hip-joint represented in Fig. 5. We are reduced, then, to this, traction loses its power when the joint is partly straightened, and pressure and counterpressure fail for the want of suitable leverage above the joint. The mechanical disadvantages thus revealed are certainly not encouraging. A ray of hope, however, is found in the reflection that the immobility is not of a hard-and-fast kind, and that, as the bad position is that which furnishes the most convenience in ordinary movements, it may be possible to accustom the patient to other habitual movements in which he will have most convenience with the limb in a good position.

The

I propose to give up the various theories which have been advanced to explain why the limb is flexed and adducted, and to find a simple and sufficient explanation in the proposition that the patient unconsciously assumes deformity because, when it is flexed and adducted, the disabled limb is less liable to painful disturbance and less in the way of the well limb, which now does almost all of the work of locomotion. limb is apparently shortened (adducted) to keep it from unnecessarily touching the ground, and the appearance of lameness is increased by the action of the well foot which violates the natural rhythm, which, as we all know, is expressed thus: one-two-one-two -one-two. As the well foot hastens forward to relieve the affected foot of the weight of the body, and prolongs its stay on the ground with the same object in view, the rhythm becomes unnatural and may be expressed thus: one-two- -one-two- -one-two.

Now, if the patient can be induced to resume the natural rhythm, in which the time is evenly divided between the two feet, he will unconsciously surrender flexion and adduction, because in that way only is it convenient or possible for him to walk in the prescribed normal rhythm of locomotion. To put it in other words: He acquires adduction or apparent shortening because he habitually keeps the affected foot on the ground less than half the time, to the destruction of natural rhythm. If he restores the natural rhythm, and keeps the foot on the ground half the time, adduction or apparent shortening will disappear. But this cannot be done during the progress of the disease without mechanical assistance. Unsymmetrical walking is nature's conservative method of keeping the affected limb off the ground as much as possible in the act of walking, in order to avoid pressure and concussion; but with an ischiatic support which keeps the heel clear of the ground, there is no reason why the affected side, splint and all, should not come to the ground as promptly and stay on the ground as long as the well side. If the patient does this and thus lets the affected side do its share of the work, it will be found that without knowing it he is reducing his flexion and adduc

tion and parting with the chief elements of his deformity, and this in accordance with the proposition above made, that the position of the limb in hip disease is that which affords the most comfort and convenience in habitual attitudes and movements.

We may glance at the comparative advantages of this method : : 1. The permanence of the result. If the deformity is once reduced in this way all that is necessary to make the result permanent is for the patient to persist in walking in correct rhythm. sist in walking in correct rhythm. If, however, a moderate deformity is reduced by forcible or operative methods, it will return if the patient maintains an unsymmetrical gait. The only exception to this would be a rare case of bony anchylosis and deformity corrected by fracture below the joint and union in a better position. 2. The early applicability of the method. Deformity may be thus reduced at the earliest stage of the disease and at the very beginning of treatment. Exceptions would be found to this in those acute periods in which the patient is averse to locomotion. 3. The late applicability of this method to patients who have recovered with deformity. Even in middle life the deformity following early hip disease may thus be reduced in an important degree.

I speak from experience when I say that by this method a great deal of the deformity attending hip disease may be removed and prevented. Success is not attained in every case, because some children can only with great difficulty be taught to walk properly, or in fact to do anything which they ought to do, especially if the parents are preoccupied or lack intelligence. But the great majority of patients are benefited in this way and readily acquire a new manner of walking, if a little of the time and attention which is given to their training in general be turned in this direction.

Progress of Medical Science.

Total Extirpation of the Rectum.-Vanderlinden and de Buck (La Flandre Méd., March 7th) claim that partial resection, or even total extirpation, of the rectum for cancer is abundantly justified where at all practicable from the point of view both of its immediate and ultimate results. They record two successful cases of this kind. Case I.-A multipara, aged thirty-one, in August, 1892, gave a history of a year and a half of pain in the lower belly, constipation, difficult defecation, grooved fæces. For a year glairy mucus, blood, and yellowish fetid sanious liquid had been passed with the fæces; marked loss of appetite and body weight. Per anum a growth was felt, ulcerated in places, extending 7 ctm. from below, and invading the whole circumference of the rectum, with its greatest thickness posteriorly. The summit of the growth was easily reached, and the whole tumor could be moved downward. The operation was performed on October 30, 1892. The dorsal position was used, with the pelvis raised, and thighs strongly flexed on the abdomen. The anus was surrounded by two short incisions, which joined in front and behind. A posterior median incision was prolonged from these to the coccyx. The anal canal and rectum were dissected out as far as 3 ctm. above the growth, where section of the bowel was made. Suture of the bowel walls to the skin wound completed the operation, which lasted an hour. The patient returned home at the end of four weeks, and three months after had gained 10 kilos. in weight, and could already retain firm stools. There has been no recurrence up to the pres ent time. The growth proved microscopically to be a lobulated epithelioma. Case II.-C. D, aged fiftytwo, married; no children. Three years' history, commencing from the climacteric, and in its details very similar to Case I. Two indurated ulcerated masses were found in the anal region. The rectum was in