title: Darier disease.
abstract: Darier disease (DD) is a type of inherited keratinizing disorder that exhibits autosomal dominant inheritance. DD is caused by the mutations of ATP2A2, which encodes an endoplasmic reticulum calcium pump, sarco/endoplasmic reticulum ATPase type 2 (SERCA2). DD often develops in childhood, persists through adolescence, and causes small papules predominantly in seborrheic areas such as the face, chest and back. Further, scales and scabs may gradually develop. DD may be accompanied by non-dermal symptoms, including psychiatric symptoms. Histologically, DD is characterized by corps ronds and grains in addition to suprabasal cleavage. There are no currently validated curative treatments available for DD, with the majority of cases treated symptomatically. Despite demonstrating efficacy in the treatment of DD, the use of oral retinoids has been limited due to the association with various adverse effects.
authors.affiliation: Department of Dermatology and Allergology, Juntendo University Graduate School of Medicine, Tokyo, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Takagi
authors.affiliation: Department of Dermatology and Allergology, Juntendo University Graduate School of Medicine, Tokyo, Japan.
authors.forename: Maya
authors.initials: M
authors.lastname: Kamijo
authors.affiliation: Department of Dermatology and Allergology, Juntendo University Graduate School of Medicine, Tokyo, Japan.
authors.forename: Shigaku
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D007644:Darier Disease / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D003937:Diagnosis, Differential; D006801:Humans; D009154:Mutation; D053498:Sarcoplasmic Reticulum Calcium-Transporting ATPases / Q000235:genetics
chemical_list: D053498:Sarcoplasmic Reticulum Calcium-Transporting ATPases; C506946:ATP2A2 protein, human
keywords: ATP2A2; Darier disease; pathogenesis; review; sarco/endoplasmic reticulum ATPase type 2