Pemphigus, Benign Familial [benigní familiární pemfigus]
topical
11
- Terms
-
Haileyho-Haileyho nemoc
Haileyova-Haileyova nemoc
pemfigus benigní familiární
-
Benign Chronic Pemphigus
Chronic Benign Familial Pemphigus
Familial Benign Chronic Pemphigus
Hailey-Hailey Disease
Persistent link
https://www.medvik.cz/link/D016506
Definition
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.
- DUI
- D016506 MeSH Browser
- CUI
- M0025199
- Previous indexing
- Pemphigus (1966-1991)
- History note
- 92
- Public note
- 92
Allowable subheadings
- BL
- blood 0
- CF
- cerebrospinal fluid 0
- CI
- chemically induced 0
- CL
- classification 0
- CO
- complications 0
- DI
- diagnosis 6
- DG
- diagnostic imaging 0
- DH
- diet therapy 0
- DT
- drug therapy 2
- EC
- economics 0
- EM
- embryology 0
- EN
- enzymology 0
- EP
- epidemiology 0
- EH
- ethnology 0
- ET
- etiology 3
- GE
- genetics 0
- HI
- history 0
- IM
- immunology 0
- ME
- metabolism 0
- MI
- microbiology 0
- MO
- mortality 0
- NU
- nursing 0
- PS
- parasitology 0
- PA
- pathology 1
- PP
- physiopathology 1
- PC
- prevention & control 0
- PX
- psychology 0
- RT
- radiotherapy 0
- RH
- rehabilitation 0
- SU
- surgery 0
- TH
- therapy 6
- UR
- urine 0
- VE
- veterinary 0
- VI
- virology 0
...
Occurrences in Medvik records