Pemphigus, Benign Familial
"Pemphigus, Benign Familial" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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.
| Descriptor ID |
D016506
|
| MeSH Number(s) |
C16.320.850.700 C17.800.827.700 C17.800.865.858
|
| Concept/Terms |
Pemphigus, Benign Familial- Pemphigus, Benign Familial
- Benign Familial Pemphigus
- Familial Pemphigus, Benign
- Benign Chronic Pemphigus
- Familial Benign Chronic Pemphigus
- Hailey-Hailey Disease
- Hailey Hailey Disease
|
Below are MeSH descriptors whose meaning is more general than "Pemphigus, Benign Familial".
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