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".
Below are MeSH descriptors whose meaning is more specific than "Pemphigus, Benign Familial".
This graph shows the total number of publications written about "Pemphigus, Benign Familial" by people in this website by year, and whether "Pemphigus, Benign Familial" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 2017 | 3 | 0 | 3 |
| 2018 | 3 | 0 | 3 |
| 2019 | 1 | 0 | 1 |
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Below are the most recent publications written about "Pemphigus, Benign Familial" by people in Profiles.
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Exacerbation of Hailey-Hailey Disease Following SARS-CoV-2 Vaccination. Acta Derm Venereol. 2021 Sep 22; 101(9):adv00554.
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Hailey-Hailey disease: a diagnostic challenge. Cutis. 2019 Mar; 103(3):157-159.
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Hailey-Hailey disease successfully treated with vitamin D oral supplementation. Dermatol Ther. 2019 01; 32(1):e12767.
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Segmental Hailey-Hailey disease of the vulva. Pediatr Dermatol. 2018 Nov; 35(6):e398-e399.
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Psoriasiform Hailey-Hailey Disease Presenting as Erythematous Psoriasiform Plaques Throughout the Body: A Case Report. Perm J. 2018; 22:17-016.
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Persisting Ulcerating Lesions After Cyclosporine Therapy for Erosions Found in Flexural Sites. Skinmed. 2017; 15(6):474-475.
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Hailey-Hailey Disease Presenting as Lichenoid Plaques on the Thigh. Skinmed. 2017; 15(5):387-388.
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Dermoscopic presentation of Hailey-Hailey disease. J Am Acad Dermatol. 2017 Feb; 76(2S1):S31-S33.