Darier Disease [Darierova nemoc]
- Terms
-
acrokeratosis verruciformis Hopf
Darierova choroba
Darierova-Whiteova choroba
dyskeratosis follicularis
dyskeratosis vegetans
dyskeratotický akantolytický epidermální névus
folikulární keratóza
Hopfův syndrom
keratosis follicularis
morbus Darier
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Acantholytic Dyskeratotic Epidermal Nevi
Acantholytic Dyskeratotic Epidermal Nevus
Acrokeratosis Verruciformis
Acrokeratosis Verruciformis of Hopf
Darier-White Disease
Darier's Disease
Hopf Disease
Keratosis Follicularis
An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the ATP2A2 gene encoding SERCA2 protein, one of the SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES. The condition is similar, clinically and histologically, to BENIGN FAMILIAL PEMPHIGUS, another autosomal dominant skin disorder. Both diseases have defective calcium pumps (CALCIUM-TRANSPORTING ATPASES) and unstable desmosomal adhesion junctions (DESMOSOMES) between KERATINOCYTES.
- DUI
- D007644 MeSH Browser
- CUI
- M0011973
- History note
- 2009 (1966)
- Public note
- 2009; see KERATOSIS FOLLICULARIS 1966-2008
Allowable subheadings
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