Darier 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.

Also Known As:

Darier's Disease; Acantholytic Dyskeratotic Epidermal Nevi; Acantholytic Dyskeratotic Epidermal Nevus; Acrokeratosis Verruciformis; Hopf 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 Diseases; Verruciformis, Acrokeratosis; Acrokeratosis Verruciformis of Hopf; Darier-White Disease; Keratosis Follicularis

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Genetic Skin Diseases: 47
    - Darier Disease: 406
Skin and Connective Tissue Diseases
- Skin Diseases: 13127
  - Keratosis: 706
    - Darier Disease: 406
  - Genetic Skin Diseases: 47
    - Darier Disease: 406

Carol Coley Selected Research

Carol Coley Research Topics

Disease

Therapy/Procedure