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Descriptor English: Pemphigus, Benign Familial
Descriptor Spanish: Pénfigo Familiar Benigno
Descriptor Portuguese: Pênfigo Familiar Benigno
Descriptor French: Pemphigus chronique bénin familial
Entry term(s): Benign Chronic Pemphigus
Benign Familial Pemphigus
Chronic Benign Familial Pemphigus
Familial Benign Chronic Pemphigus
Familial Pemphigus, Benign
Hailey Hailey Disease
Hailey-Hailey Disease
Tree number(s): C16.320.850.700
C17.800.827.700
C17.800.865.858
RDF Unique Identifier: https://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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Pemphigus (1966-1991)
Public MeSH Note: 92
History Note: 92
DeCS ID: 29899
Unique ID: D016506
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1992/01/01
Date of Entry: 1991/01/03
Revision Date: 2019/02/22
Pemphigus, Benign Familial - Preferred
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.
Preferred term Pemphigus, Benign Familial
Entry term(s) Benign Chronic Pemphigus
Benign Familial Pemphigus
Chronic Benign Familial Pemphigus
Familial Benign Chronic Pemphigus
Familial Pemphigus, Benign
Hailey Hailey Disease
Hailey-Hailey Disease



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