Darierwhite s disease, follicular dyskeratosis, genodermatosis. Dariers disease dd, also known as keratosis follicularis or darierwhite disease, is a rare disorder of keratinization. The darierwhite disease is an autosomal dominant genodermatosis with full expression in adulthood. The most important morbidities of darier disease are the psychosocial con. This illustrates the intense itching these patients get. Darierwhites disease, follicular dyskeratosis, genodermatosis.
Abstract darierwhite disease is a dermatosis reported in 1889, is autosomic dominant and is characterized by hiperkeratotic papules localized mainly at seborrheic regions as well as ungueal abnormalities. All structured data from the file and property namespaces is available under the creative commons cc0 license. The disease is caused by a lossoffunction mutation in the atp2a2 gene on chromosome 12q2324 that encodes the. Read dariers disease associated with basal cell carcinomas, dermatologic surgery on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at. Dyskeratosis follicularis darierwhite disease is a rare autosomal dominant transmitted disease due to an alteration of keratinization in epidermis, nails and. See more of medicina humana en imagenes on facebook. The patients generally are scratching interminably.
Dariers disease, also known as keratosis follicularis, was first described by morrow in 1886 and subsequently in 1889 by darier and white in an independent manner. It is a very hard disease to treat, and i do not know of a medication that will adequately bring it under control, other than oral or topical steroids. Files are available under licenses specified on their description page. Associazione rete malattie rare onlus retemalattierare.
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