Diseases

Benign Familial Pemphigus

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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 Record

Classification

Broader headings

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MeSH Record

Synonyms

7 entry terms
  • Benign Chronic Pemphigus
  • Chronic Benign Familial Pemphigus
  • Familial Benign Chronic Pemphigus
  • Hailey-Hailey Disease
  • Pemphigus, Benign Familial
  • Familial Pemphigus, Benign
  • Hailey Hailey Disease

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

92

MeSH Record

Previous Indexing

  • Pemphigus (1966-1991)

MeSH Hierarchy

Tree Numbers

  1. C16w.320w.850w.700w
  2. C17.800w.827w.700w
  3. C17.800w.865w.858w

AMA Style

References

  1. National Library of Medicine. Benign Familial Pemphigus. Medical Subject Headings (MeSH). 2026. Unique ID D016506. http://id.nlm.nih.gov/mesh/2026/D016506
  2. Benign Familial Pemphigus. In: Wikipedia. https://en.wikipedia.org/wiki/Hailey%E2%80%93Hailey_disease
  3. Benign Familial Pemphigus. In: Wikidata. https://www.wikidata.org/wiki/Q863861