JLE

European Journal of Dermatology

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Type 2 segmental Darier disease Volume 9, numéro 6, September 1999

Auteurs
Department of Dermatology, University of Marburg, Deutschhausstrasse 9, 35033 Marburg, Germany.
  • Page(s) : 449-51
  • Année de parution : 1999

Darier disease is an autosomal dominant skin disorder (OMIM 124 200) caused by mutations in the ATP2A2 gene that encodes an intracellular Ca2+ pump [1]. This defect of the Ca2+-signalling pathway results in a disturbed adhesion of keratinocytes that gives rise to characteristic histopathological changes in the form of acantholysis and dyskeratosis [2]. Clinical features consist of disseminated brownish and pruritic papules [...]