The peeling skin syndrome - 07/10/17
Résumé |
A unique form of congenital ichthyosis in two unrelated patients is described and characterized histologically by separation of the epidermis between the stratum comeum and the stratum granulosum. The clinical history, genetics, serially performed skin biopsies, and biochemical studies are reviewed. This form of lchthyosls is different from previously described entities. Lifelong peeling of the general body epidermis, pruntus, short stature, easily removed anagen hairs, and the ability to easily mechanically separate stratum corneum from the rest of the epidermis characterize the syndrome. In two families with this disorder, autosomal recessive inheritance is suggested. A low plasma tryptophan level was present in two patients with this disease. This inherited disorder of the epidermis was first described in 1924 before the genetics and histology of ichthyosis were extensively studied and is a distinct genetic and clinical entity to be considered in unusual cases of ichthyosis.
Le texte complet de cet article est disponible en PDF.* | Publication No 88 from the Dermatological Research Laboratories at Duke University Medical Center |
Supported in part by grants 2 RO1 AM 17253 and 5 T32 AM 07093 from the National Institutes of Health, and Dr Lowell A Goldsmith was the recipient of Research Career and Development Award 5 KO4 AM 00008 from the National Institutes of Health. The patients were hospitalized in the Duke University Clinical Research Unit, supported by grant RR-30 from the National Institutes of Health. |
Vol 7 - N° 5
P. 606-613 - novembre 1982 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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