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DARIER-WHITE DISEASE
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DeCS
Descriptor
English
:
Darier Disease
Descriptor
Spanish
:
Enfermedad de Darier
Descriptor
Portuguese
:
Doença de Darier
Synonyms
English
:
Acrokeratosis Verruciformis of Hopf
Darier-White Disease
Keratosis Follicularis
Tree Number:
C16.320.850.190
C17.800.428.275
C17.800.827.190
Definition
English
:
An autosomal dominantly inherited skin disorder characterized by warty malodorous papules that coalesce into plaques. It is caused by mutations in the ATP2A2 gene encoding SERCA2 protein, one of the
SARCOPLASMIC RETICULUM CALCIUM-TRANSPORTING ATPASES
. The condition is
similar
, clinically and histologically, to BENIGN FAMILIAL
PEMPHIGUS
, another autosomal dominant skin disorder. Both diseases have defective
calcium
pumps (
CALCIUM-TRANSPORTING ATPASES
) and unstable desmosomal adhesion junctions (
DESMOSOMES
) between
KERATINOCYTES.
History Note
English
:
2009 (1966)
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:
7811
Unique Identifier:
D007644
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS