| 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): |
Benign Chronic Pemphigus Benign Familial Pemphigus Chronic Benign Familial Pemphigus Familial Benign Chronic Pemphigus Familial Pemphigus, Benign Hailey Hailey Disease Hailey-Hailey Disease |
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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 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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| Previous Indexing: |
Pemphigus (1966-1991) |
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| Public MeSH Note: | 92 |
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| History Note: | 92 |
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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, Benign Familial
- Preferred
| Concept UI |
M0025199 |
| 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. |
| Preferred term | Pemphigus, Benign Familial |
| Entry term(s) |
Benign Chronic Pemphigus Benign Familial Pemphigus Chronic Benign Familial Pemphigus Familial Benign Chronic Pemphigus Familial Pemphigus, Benign Hailey Hailey Disease Hailey-Hailey Disease |
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