Pemphigus, Benign Familial

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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.

MeSH information

Definition | Details | More General Concepts | Related Concepts | More Specific Concepts

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 Familial Benign Chronic Pemphigus Hailey-Hailey Disease Hailey Hailey Disease Benign Chronic Pemphigus

Below are MeSH descriptors whose meaning is more general than "Pemphigus, Benign Familial".

Diseases [C]
Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
Genetic Diseases, Inborn [C16.320]
Skin Diseases, Genetic [C16.320.850]
Pemphigus, Benign Familial [C16.320.850.700]
Skin and Connective Tissue Diseases [C17]
Skin Diseases [C17.800]
Skin Diseases, Genetic [C17.800.827]
Pemphigus, Benign Familial [C17.800.827.700]
Skin Diseases, Vesiculobullous [C17.800.865]
Pemphigus, Benign Familial [C17.800.865.858]

Below are MeSH descriptors whose meaning is related to "Pemphigus, Benign Familial".

Below are MeSH descriptors whose meaning is more specific than "Pemphigus, Benign Familial".

publications

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This graph shows the total number of publications written about "Pemphigus, Benign Familial" by people in UAMS Profiles by year, and whether "Pemphigus, Benign Familial" was a major or minor topic of these publications.

Bar chart showing 2 publications over 2 distinct years, with a maximum of 1 publications in 2016 and 2021

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