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| Descriptor en español: |
Pénfigo Familiar Benigno
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| Descriptor en inglés: | Pemphigus, Benign Familial | ||||||
| Descriptor en portugués: | Pênfigo Familiar Benigno | ||||||
| Descriptor en francés: | Pemphigus chronique bénin familial | ||||||
| Término(s) alternativo(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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| Código(s) jeráquico(s): |
C16.320.850.700 C17.800.827.700 C17.800.865.858 |
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| Identificador Único RDF: | https://id.nlm.nih.gov/mesh/D016506 | ||||||
| Nota de alcance: | 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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| Calificadores permitidos: |
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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| Indexación anterior: |
Pemphigus (1966-1991) |
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| Nota Pública de MeSH: | 92 |
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| Nota de historia: | 92 |
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| Identificador de DeCS: | 29899 | ||||||
| ID del Descriptor: | D016506 | ||||||
| Documentos indizados en la Biblioteca Virtual de Salud (BVS): | Haga clic aquí para acceder a los documentos de la BVS | ||||||
| Fecha de establecimiento: | 01/01/1992 | ||||||
| Fecha de entrada: | 03/01/1991 | ||||||
| Fecha de revisión: | 22/02/2019 |
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Pemphigus, Benign Familial
- Concepto preferido
| UI del concepto |
M0025199 |
| Nota de alcance | 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. |
| Término preferido | Pemphigus, Benign Familial |
| Término(s) alternativo(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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