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Congen1ta

skin, birth and alive

CONGEN1TA (aKACEA) (Keratosis diffusa congenita) This infantile affection was first described in 1792 and again later under a variety of different names. It is congenital and consists of an enormous deposit of sebum and corneal masses upon the skin of the newborn infant. The affection is comparatively rare (Riecke, 1901, 51 cases), and OCCUN at birth in weak infants. Usually the child dies in a week or sooner, although in light cases they may be kept alive for a longer period. The skin consists of large horny masses broken up by fissures and furrows, and seems too tight for the body. The mouth and its angles are distorted, the curves obliterated, ectropia and deformi ties of the nose develop, and the folds of the skin are absent. The disease is generally developed in utero (in the fourth month) and the ehild dies from inanition, being unable to suckle. In lighter eases where the covering is more parchnicutlike, the attempt to keep the ehild alive for a longer periotl is successful. Riecke dis tinguishes between three forms: (1) true ichthyosis congenita (severe eases developed in utero; die within one to four days after birth); (2) ichtliyosis congenita larvata (milder cases or those which involve the skin only partially, developed entirely or nearly so at birth; children remain alive for a longer period); (3) ieldhyosis congenita tarda (birth normal, appearance of the disease at a later period; children sometimes remain alive).

Pathological Anatomy.—There is considerable thickening of the corneal layer (up to 500 A as against 34 to 47 of the normal skin, which is aug mented by new formation in the rete, and rapid cornifieation producing diffuse keratoma. The cutis is normal (Kyber, Wassmuth, Riecke, Neumann).

Etiology.—The etiology is still completely unknown. Prognosis.—The prognosis is generally fatal, except in light ea.ses.

Treatment.—Baths, improvement of nutrition, embrocation with mild oint ments, would seem to be the best measures.