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Pemphigus, Benign Familial MeSH Descriptor Data 2021


MeSH Heading
Pemphigus, Benign Familial
Tree Number(s)
C16.320.850.700
C17.800.827.700
C17.800.865.858
Unique ID
D016506
RDF Unique Identifier
http://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.
Entry Term(s)
Benign Chronic Pemphigus
Chronic Benign Familial Pemphigus
Familial Benign Chronic Pemphigus
Hailey-Hailey Disease
Previous Indexing
Pemphigus (1966-1991)
Public MeSH Note
92
History Note
92
Date Established
1992/01/01
Date of Entry
1991/01/03
Revision Date
2019/02/22
Pemphigus, Benign Familial Preferred
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