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Case Reports
April 1938

XERODERMA PIGMENTOSUM WITH MENTAL DEFICIENCY: A REPORT OF TWO CASES

Am J Dis Child. 1938;55(4):784-791. doi:10.1001/archpedi.1938.01980100120008
Abstract

Xeroderma pigmentosum is a comparatively rare disease which was first described by Kaposi.1 It is of unknown etiology and probably congenital in origin. Consanguinity of the parents is said to be a predisposing factor. The onset of the disease occurs characteristically during the first year of life. The skin is apparently hypersensitive to light rays of wavelengths between 280 and 310 millimicrons.2 The pathologic condition of the skin shows three stages:3 (1) erythroderma, with mottling and hyperemia, slight puffiness and roughening, (2) pigmentation, with freckle formation, scaling and the formation of transient flat warts, and (3) the appearance of nonmetastasizing epitheliomas, cutaneous atrophy and late contractures of the eyelids, nose and mouth. The course is progressive but usually shows periods of quiescence. The duration of life is, as a rule, not beyond twenty-one years. Death generally results from inanition.

Most of the cases of this condition have

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