MeSH Browser

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

Allowable Qualifiers: blood (BL)

cerebrospinal fluid (CF)

chemically induced (CI)

classification (CL)

complications (CO)

diagnosis (DI)

diagnostic imaging (DG)

diet therapy (DH)

drug therapy (DT)

economics (EC)

embryology (EM)

enzymology (EN)

epidemiology (EP)

ethnology (EH)

etiology (ET)

genetics (GE)

history (HI)

immunology (IM)

metabolism (ME)

microbiology (MI)

mortality (MO)

nursing (NU)

parasitology (PS)

pathology (PA)

physiopathology (PP)

prevention & control (PC)

psychology (PX)

radiotherapy (RT)

rehabilitation (RH)

surgery (SU)

therapy (TH)

urine (UR)

veterinary (VE)

virology (VI)

Concept UI: M0025199
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.
Terms: Pemphigus, Benign Familial Preferred Term
Term UI T049368
Date01/01/1999
LexicalTag NON
ThesaurusID; Chronic Benign Familial Pemphigus
Term UI T000948176
Date11/05/2018
LexicalTag NON
ThesaurusID NLM (2020); Benign Chronic Pemphigus
Term UI T769478
Date04/15/2010
LexicalTag NON
ThesaurusID; Familial Benign Chronic Pemphigus
Term UI T049366
Date08/23/1990
LexicalTag NON
ThesaurusID NLM (1992); Hailey-Hailey Disease
Term UI T049367
Date08/23/1990
LexicalTag EPO
ThesaurusID

Pemphigus, Benign Familial MeSH Descriptor Data 2024