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| Descriptor English: | Darier Disease | ||||||
| Descriptor Spanish: |
Enfermedad de Darier
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| 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 |
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| Tree number(s): |
C16.320.850.190 C17.800.428.275 C17.800.827.190 |
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| 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. |
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| 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 |
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| Public MeSH Note: | 2009; see KERATOSIS FOLLICULARIS 1966-2008 |
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| History Note: | 2009 (1966) |
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| Entry Version: | DARIER DIS |
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| 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 |
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Darier Disease
- Preferred
Acrokeratosis Verruciformis of Hopf
- Related but not broader or narrower
Acantholytic Dyskeratotic Epidermal Nevus
- Related but not broader or narrower
| 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 |
| 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 |
| Concept UI |
M0513150 |
| Preferred term | Acantholytic Dyskeratotic Epidermal Nevus |
| Entry term(s) |
Acantholytic Dyskeratotic Epidermal Nevi |
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