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Darier Disease MeSH Descriptor Data 2022


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 Established
2009/01/01
Date of Entry
1999/01/01
Revision Date
2015/06/18
Darier Disease Preferred
Acrokeratosis Verruciformis of Hopf Related
Acantholytic Dyskeratotic Epidermal Nevus Related
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