The peeling skin syndrome.

Published

Journal Article

A unique form of congenital ichthyosis in two unrelated patients is described and characterized histologically by separation of the epidermis between the stratum corneum and the stratum granulosum. The clinical history, genetics, serially performed skin biopsies, and biochemical studies are reviewed. This form of ichthyosis is different from previously described entities. Lifelong peeling of the general body epidermis, pruritus, 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 as 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.

Full Text

Duke Authors

Cited Authors

  • Levy, SB; Goldsmith, LA

Published Date

  • November 1, 1982

Published In

Volume / Issue

  • 7 / 5

Start / End Page

  • 606 - 613

PubMed ID

  • 7142468

Pubmed Central ID

  • 7142468

International Standard Serial Number (ISSN)

  • 0190-9622

Digital Object Identifier (DOI)

  • 10.1016/s0190-9622(82)70140-9

Language

  • eng

Conference Location

  • United States