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Darier Disease and Related Disorders


Authors: Z. Plzáková 1;  J. Štork 1;  M. Šlajsová 1
Authors‘ workplace: Dermatovenerologická klinika 1. LF UK a VFN, Praha přednosta prof. MUDr. Jiří Štork, CSc. 1
Published in: Čes-slov Derm, 85, 2010, No. 6, p. 309-316
Category: Reviews (Continuing Medical Education)

Overview

Darier disease and Hailey-Hailey disease are autosomal dominant dermatoses caused by dysfunction of intracellular transport of Ca2+ ions leading to defects of keratinocyte adhesion followed by suprabasal acantholysis and dyskeratosis. Clinical lesions of various extent are usually worsened by influence of mechanical and thermal irritating factors. Etiopathogenesis of Grover disease has not been fully disclosed yet. Article reviews the typical and less common disease manifestations as well as contemporary therapeutic options.

Key words:
Darier disease – Hailey-Hailey disease – Grover disease – acantholytic dyskeratosis – Ca2+ ATPase – retinoids


Sources

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