Descriptors Found: 1
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 1 / 1 DeCS     
Descriptor English:   Pemphigus, Benign Familial 
Descriptor Spanish:   Pénfigo Familiar Benigno 
Descriptor Portuguese:   Pênfigo Familiar Benigno 
Synonyms English:   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:   C16.320.850.700
Definition English:   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. 
History Note English:   92 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications DI diagnosis
DG diagnostic imaging DH diet therapy
DT drug therapy EC economics
EM embryology EN enzymology
EP epidemiology EH ethnology
ET etiology GE genetics
HI history IM immunology
ME metabolism MI microbiology
MO mortality NU nursing
PS parasitology PA pathology
PP physiopathology PC prevention & control
PX psychology RT radiotherapy
RH rehabilitation SU surgery
TH therapy UR urine
VE veterinary VI virology
Record Number:   29899 
Unique Identifier:   D016506 

Occurrence in VHL: