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| Descriptor English: | Pemphigus, Benign Familial | ||||||
| Descriptor Spanish: |
Pénfigo Familiar Benigno
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| Descriptor Portuguese: | Pênfigo Familiar Benigno | ||||||
| Descriptor French: | Pemphigus chronique bénin familial | ||||||
| Entry term(s): |
Dermatose acantholytique familiale Maladie de Hailey et Hailey Maladie de Hailey-Hailey Maladie de Hugh-Hailey Pemphigus bénin chronique familial |
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| Tree number(s): |
C16.320.850.700 C17.800.827.700 C17.800.865.858 |
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| RDF Unique Identifier: | https://id.nlm.nih.gov/mesh/D016506 | ||||||
| Scope note: | An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and BULLAE mainly on the neck, axillae, and groin. Mutations in the ATP2C1 gene (encoding the secretory pathway Ca2++/Mn2++ ATPase 1 (SPCA1)) cause this disease. It is clinically and histologically similar to DARIER DISEASE - both have abnormal, unstable DESMOSOMES between KERATINOCYTES and defective CALCIUM-TRANSPORTING ATPASES. It is unrelated to PEMPHIGUS VULGARIS though it closely resembles that disease. |
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| Allowable Qualifiers: |
BL sang CF liquide cérébrospinal CI induit chimiquement CL classification CO complications DG imagerie diagnostique DH diétothérapie DI diagnostic DT traitement médicamenteux EC économie EH ethnologie EM embryologie EN enzymologie EP épidémiologie ET étiologie GE génétique HI histoire IM immunologie ME métabolisme MI microbiologie MO mortalité NU soins infirmiers PA anatomopathologie PC prévention et contrôle PP physiopathologie PS parasitologie PX psychologie RH rééducation et réadaptation RT radiothérapie SU chirurgie TH thérapie UR urine VE médecine vétérinaire VI virologie |
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| DeCS ID: | 29899 | ||||||
| Unique ID: | D016506 | ||||||
| Documents indexed in the Virtual Health Library (VHL): | Click here to access the VHL documents | ||||||
| Date Established: | 1992/01/01 | ||||||
| Date of Entry: | 1991/01/03 | ||||||
| Revision Date: | 2019/02/22 |
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Pemphigus chronique bénin familial
- Preferred
| Concept UI |
M0025199 |
| Preferred term | Pemphigus chronique bénin familial |
| Entry term(s) |
Dermatose acantholytique familiale Maladie de Hailey et Hailey Maladie de Hailey-Hailey Maladie de Hugh-Hailey Pemphigus bénin chronique familial |
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