Type 1 segmental manifestation of Hailey-Hailey disease.

Publication/Presentation Date

10-1-2003

Abstract

Two types of mosaic manifestations can be distinguished in autosomal dominant skin disorders. A type-1 mosaicism reflects a localized postzygotic mutation in an otherwise normal embryo. This mutation leads to a localized population of heterozygous cells, resulting in segmental disease. In contrast, a type-2 mosaicism represents a postzygotic mutation eliminating the normal allele at a gene locus, for which the embryo carries a dominant heterozygous germline mutation. The corresponding phenotype is characterized by segmental lesions superimposed on "classical" disease. The authors describe the clinical and histopathologic aspects of the first case of type-1 segmental manifestation of Hailey-Hailey disease.

Volume

49

Issue

4

First Page

712

Last Page

714

ISSN

0190-9622

Disciplines

Medicine and Health Sciences

PubMedID

14512922

Department(s)

Department of Pathology and Laboratory Medicine

Document Type

Article

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