Darier Disease


Acantholytic Dyskeratotic Epidermal Nevi

Acantholytic Dyskeratotic Epidermal Nevus

Acrokeratosis Verruciformis

Acrokeratosis Verruciformis of Hopf

Darier White Disease

Darier's Disease

Darier-White Disease

Darier-White Diseases

Dariers Disease

Disease, Darier

Disease, Darier's

Disease, Darier-White

Disease, Hopf

Diseases, Darier-White

Diseases, Hopf

Hopf Acrokeratosis Verruciformis

Hopf Disease

Hopf Diseases

Keratosis Follicularis

Verruciformis, Acrokeratosis

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.