Because of a lapse in government funding, the information on this website may not be up to date, transactions submitted via the website may not be processed, and the agency may not be able to respond to inquiries until appropriations are enacted. The NIH Clinical Center (the research hospital of NIH) is open. For more details about its operating status, please visit cc.nih.gov. Updates regarding government operating status and resumption of normal operations can be found at opm.gov.

NLM Logo

Darier Disease MeSH Descriptor Data 2026


MeSH Heading
Darier Disease
Tree Number(s)
C16.320.850.190
C17.800.428.275
C17.800.827.190
Unique ID
D007644
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D007644
Scope Note
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.
Entry Version
DARIER DIS
Entry Term(s)
Acantholytic Dyskeratotic Epidermal Nevi
Acantholytic Dyskeratotic Epidermal Nevus
Acrokeratosis Verruciformis
Acrokeratosis Verruciformis of Hopf
Darier's Disease
Darier-White Disease
Hopf Disease
Keratosis Follicularis
Public MeSH Note
2009; see KERATOSIS FOLLICULARIS 1966-2008
History Note
2009 (1966)
Date Introduced
2009/01/01
Last Updated
2015/06/18
Darier Disease Preferred
Acrokeratosis Verruciformis of Hopf Related
Acantholytic Dyskeratotic Epidermal Nevus Related
page delivered in 0.147s