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XERODERMA PIGMENTOSUM WITH MENTAL DEFICIENCY

XERODERMA PIGMENTOSUM WITH MENTAL DEFICIENCY 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 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American journal of diseases of children American Medical Association

XERODERMA PIGMENTOSUM WITH MENTAL DEFICIENCY

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References (14)

Publisher
American Medical Association
Copyright
Copyright © 1938 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.
ISSN
0096-8994
eISSN
1538-3628
DOI
10.1001/archpedi.1938.01980100120008
Publisher site
See Article on Publisher Site

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

Journal

American journal of diseases of childrenAmerican Medical Association

Published: Apr 1, 1938

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