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Darier's Disease Acrokeratosis Verruciformis Diseases, Hopf Acrokeratosis Verruciformis of Hopf Keratosis Follicularis Diseases, Darier-White Disease, Hopf Darier White Disease Hopf Disease Darier-White Diseases Disease, Darier Hopf Diseases Acantholytic Dyskeratotic Epidermal Nevus Darier-White Disease Hopf Acrokeratosis Verruciformis Dariers Disease Disease, Darier's Disease, Darier-White Verruciformis, Acrokeratosis Acantholytic Dyskeratotic Epidermal Nevi
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An alleleic variant of Darier's disease. 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.
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