Skin Diseases of Children/Ichthyosis

by George Henry Fox

Plate V.

Ichthyosis

From the collection or photographs of

Dr. George Henry Fox.

ICHTHYOSIS.

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Ichthyosis is a deformity or an imperfect development rather than a disease of the skin. Like all deformities, it is very apt to be hereditary, affecting one or more members of a family, and often skipping a generation. It is characterized by a marked deficiency of the normal cutaneous secretions and a tendency to the formation of a dry, scaly surface, which suggested the name of "fish-skin disease." It is sometimes congenital, may indeed develop in utero, but often does not manifest itself until several months after birth. In rare instances it may develop late in life. Though always improved by judicious treatment, it may be considered as one of the incurable dermatoses, since the peculiar character of the skin cannot be changed.

There are various degrees of severity in which ichthyosis may manifest itself, and different names applied to these have led to some confusion as to their true character. For instance, the term xeroderma, or "parchment skin," has been used unnecessarily to designate a very mild form of ichthyosis in which the skin presents a dry, mealy appearance, especially apt to be noticed in children with whom bathing is a rare luxury. In some cases this slight ichthyotic tendency disappears in time, and sometimes appears in later life as an acquired condition. It should be borne in mind that as the hair in some children is preternaturally dry and in others unusually oily, and that as this condition may vary in the same individual according to the state of health, so the degree of natural oiliness or dryness of the skin may vary greatly in individuals and in accordance with season and general condition. A skin which is notably dry and with a tendency to chap or roughen might be regarded as xerodermatous or ichthyotic in a very slight degree.

In ichthyosis mitis (or simplex) the characteristic features of the affection are well developed. The skin is dry, and perspiration is slight even in warm weather. Upon the extensor aspect of the extremities the epidermis presents often a peculiar serpentine appearance, resulting from the cracking of the horny layer into polygonal plates, which in time assume a dirty gray or greenish tint. These plates adhere by the central portion,

Fig. 17.—Ichthyosis.

while the margin tends to separate from the underlying skin. Around the elbows and knees the natural wrinkles are greatly intensified, and the breaking of the horny epidermis occasions a large number of concentric, whitish lines separating rows of warty elevations. As a rule the flexures of the joints, axillæ, groins, genitals, palms, and soles are but slightly if at all affected. The face usually presents a characteristic appearance, the cheeks being roughened or chapped, the eyelids stiffened and drawn into a condition bordering on ectropion, the lips dry, and the oral commissures more or less wrinkled. The hair is usually dry and sparse upon the scalp, and may be entirely absent upon those portions of the trunk and extremities where a slight growth is usually present. The nails are apt to be dry and brittle.

In cases of ichthyosis of a severe type in which no treatment is instituted and the ordinary use of soap and water is neglected, the epidermis becomes broken into small plates, which increase in thickness until the affected skin upon the extensor aspect of

Fig. 18—Ichthyosis with broken epidermis.

the extremities is covered with more or less conical, blackish masses and resembles the bark of a tree. To this condition the term ichthyosis hystrix, or "porcupine skin," may be applied. It should be borne in mind, however, that this term is often used to designate a linear warty condition occurring in parallel streaks, usually upon one side of the body (nævus verrucosus), and which has no clinical relation to ordinary ichthyosis.

The severest form of the disease is one which develops in utero and is frequently fatal shortly after birth. This has been described as "diffuse congenital keratoma," "harlequin fœtus," etc. Dr. George G. Wheelock in a report of a case^[1] states that the forceps were applied through the os and the child was with considerable difficulty extracted, there seeming to be a total lack of lubrication and consequently great friction between the fœtal and maternal parts. As the head was born a thick plate of skin two inches square was detached and escaped with the head. At first the child had the appearance of a dead fœtus with macerated epidermis, but shortly it began to breathe and to cry feebly. Its appearance was horrible in the extreme. It was covered from head to foot with a skin like leather, deeply fissured and broken up into plates like an alligator or an armadillo. Many of the plates were separated from the true skin, which was of a bright strawberry color. After birth the dried

Fig. 19.—Ichthyosis with fine scales.

skin became of a bright chrome yellow, and the plates were more and more detached by the motions of the child, which lived only six hours. Another case has been reported of a woman who had three healthy children by her first husband and three ichthyotic fœtuses by her second. Cases have also been reported of this severe form of the disease developing after birth.

Occasionally one of these cases of severe congenital ichthyosis may survive. A youth known to fame as the "Alligator Boy," and exhibited in a dime museum some years ago, was described by the writer in the Journal of Cutaneous and Venereal Diseases, April, 1884. The eruption, contrary to the rule, was most marked upon the trunk, the epidermis being broken by movements of the body into polygonal, horny plates of varying size and of a dirty-yellowish hue. In winter the skin upon the trunk was smooth, though thickened and horny in character. In the spring this horny integument was wont to crack and present the characteristic alligator appearance,

Fig 20.—Polygonal plates of ichthyosis.

while the hair of the head was almost entirely shed. At birth, according to Dr. F. Kennedy, the child presented a most extraordinary appearance. The skin was smooth, as if polished, and of a deep-red color. It was impossible for the child to make use of its facial muscles in its efforts to cry or suck. After a few days fissures occurred and armor-like scales were formed, which gradually peeled off. This case was evidently of the same nature as those reported by Wheelock, Kyber, and others, but not so severe as to cause death.

Heredity is the only cause which can be suggested for the occurrence of ichthyosis, but often the parents and grand-parents are said to have been free from this or any other form of skin disease. The sexes are about equally disposed to the affection.

The treatment of ichthyosis is of necessity palliative, as the cause cannot be removed, but even in the severest cases the skin can be softened and restored temporarily to an almost or quite normal condition. To maintain this condition a considerable amount of persistent daily lubrication of the skin is required. The use of fatty articles of food, such as cream, eggs, etc., is to be recommended; and cod-liver oil will often prove serviceable, although it may not be necessary to use it internally, externally, and eternally, as has been suggested. Indeed, for local application the fats and oils do not generally prove as agreeable and effective as does diluted glycerin. A half-ounce of this added to a pint of rose water (or rainwater) makes an excellent lotion, which can be readily rubbed over the extremities, or whole body if necessary, every night and morning. When the disease is complicated by eczema, as often happens, a soothing ointment or paste is called for.

1. Illustrated Quarterly of Medicine and Surgery, July, 1882.