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.

Descriptor ID	D007644
MeSH Number(s)	C16.320.850.190 C17.800.428.275 C17.800.827.190
Concept/Terms	Darier Disease Darier Disease Disease, Darier Darier's Disease Dariers Disease Disease, Darier's Keratosis Follicularis Darier-White Disease Darier White Disease Darier-White Diseases Disease, Darier-White Diseases, Darier-White Acrokeratosis Verruciformis of Hopf Acrokeratosis Verruciformis of Hopf Hopf Acrokeratosis Verruciformis Hopf Disease Disease, Hopf Diseases, Hopf Hopf Diseases Acrokeratosis Verruciformis Verruciformis, Acrokeratosis Acantholytic Dyskeratotic Epidermal Nevus Acantholytic Dyskeratotic Epidermal Nevus Acantholytic Dyskeratotic Epidermal Nevi

Below are MeSH descriptors whose meaning is more general than "Darier Disease".

Below are MeSH descriptors whose meaning is related to "Darier Disease".

Below are MeSH descriptors whose meaning is more specific than "Darier Disease".