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PEMPHIGUS, BENIGN FAMILIAL
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DeCS
Descriptor
English
:
Pemphigus, Benign Familial
Descriptor
Spanish
:
Pénfigo Familiar Benigno
Descriptor
Portuguese
:
Pênfigo Familiar Benigno
Synonyms
English
:
Familial Benign Chronic Pemphigus
Hailey-Hailey Disease
Tree Number:
C16.320.850.700
C17.800.827.700
C17.800.865.858
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.
See Related
English
:
Pemphigus
History Note
English
:
92
Allowable Qualifiers
English
:
BL
blood
CF
cerebrospinal fluid
CI
chemically induced
CL
classification
CO
complications
DI
diagnosis
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
RA
radiography
RI
radionuclide imaging
RT
radiotherapy
RH
rehabilitation
SU
surgery
TH
therapy
US
ultrasonography
UR
urine
VE
veterinary
VI
virology
Record Number:
29899
Unique Identifier:
D016506
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS