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.
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".
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 Harvard Catalyst Profiles by year, and whether "Pemphigus, Benign Familial" was a major or minor topic of these publication.
To see the data from this visualization as text,
click here.
| Year | Major Topic | Minor Topic | Total |
|---|
| 1997 | 1 | 0 | 1 |
| 2001 | 1 | 0 | 1 |
| 2004 | 1 | 1 | 2 |
| 2013 | 0 | 1 | 1 |
| 2017 | 1 | 0 | 1 |
| 2018 | 1 | 0 | 1 |
Below are the most recent publications written about "Pemphigus, Benign Familial" by people in Profiles.
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Naltrexone for the Treatment of Darier and Hailey-Hailey Diseases. J Cutan Med Surg. 2019 Jul/Aug; 23(4):453-454.
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Variable Response to Naltrexone in Patients With Hailey-Hailey Disease. JAMA Dermatol. 2018 03 01; 154(3):362-363.
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Treatment of Hailey-Hailey disease with botulinum toxin. Australas J Dermatol. 2018 Aug; 59(3):229-231.
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Remission of refractory benign familial chronic pemphigus (hailey-hailey disease) with the addition of systemic cyclosporine. J Cutan Med Surg. 2015 Mar-Apr; 19(2):163-6.
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The secretory pathway calcium ATPase PMR-1/SPCA1 has essential roles in cell migration during Caenorhabditis elegans embryonic development. PLoS Genet. 2013 May; 9(5):e1003506.
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Familial benign chronic pemphigus (Hailey-Hailey disease). Dermatol Online J. 2009 Aug 15; 15(8):15.
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Are patients with Darier and Haily-Haily diseases susceptible to Alzheimer's disease? A theory based on abnormal intraneuronal Ca2+ homeostasis. J Alzheimers Dis. 2009; 16(3):521-3.
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What is your diagnosis? Benign familial chronic pemphigus. Cutis. 2005 Apr; 75(4):208, 210-12.
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Expression of Hailey-Hailey disease mutations in yeast. J Invest Dermatol. 2004 Dec; 123(6):1192-4.
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Functional expression of heterologous proteins in yeast: insights into Ca2+ signaling and Ca2+-transporting ATPases. Am J Physiol Cell Physiol. 2004 Sep; 287(3):C580-9.