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Descriptor English: Darier Disease
Descriptor Spanish: Enfermedad de Darier
Descriptor Portuguese: Doença de Darier
Descriptor French: Maladie de Darier
Entry term(s): Acantholytic Dyskeratotic Epidermal Nevi
Acantholytic Dyskeratotic Epidermal Nevus
Acrokeratosis Verruciformis
Acrokeratosis Verruciformis of Hopf
Darier White Disease
Darier's 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 Disease
Hopf Diseases
Keratosis Follicularis
Verruciformis, Acrokeratosis
Tree number(s): C16.320.850.190
C17.800.428.275
C17.800.827.190
RDF Unique Identifier: https://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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Public MeSH Note: 2009; see KERATOSIS FOLLICULARIS 1966-2008
History Note: 2009 (1966)
Entry Version: DARIER DIS
DeCS ID: 7811
Unique ID: D007644
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2009/01/01
Date of Entry: 1999/01/01
Revision Date: 2015/06/18
Darier Disease - Preferred
Concept UI M0011973
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.
Preferred term Darier Disease
Entry term(s) Darier White Disease
Darier's Disease
Darier-White Disease
Darier-White Diseases
Dariers Disease
Disease, Darier
Disease, Darier's
Disease, Darier-White
Diseases, Darier-White
Keratosis Follicularis
Acrokeratosis Verruciformis of Hopf - Related but not broader or narrower
Concept UI M0513138
Scope note An alleleic variant of Darier's disease.
Preferred term Acrokeratosis Verruciformis of Hopf
Entry term(s) Acrokeratosis Verruciformis
Disease, Hopf
Diseases, Hopf
Hopf Acrokeratosis Verruciformis
Hopf Disease
Hopf Diseases
Verruciformis, Acrokeratosis
Acantholytic Dyskeratotic Epidermal Nevus - Related but not broader or narrower
Concept UI M0513150
Preferred term Acantholytic Dyskeratotic Epidermal Nevus
Entry term(s) Acantholytic Dyskeratotic Epidermal Nevi



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