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EPIDERMOLYSIS BULLOSA LETALIS
Descriptors Found:
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DeCS
Descriptor
English
:
Epidermolysis Bullosa, Junctional
Descriptor
Spanish
:
Epidermólisis Ampollosa de la Unión
Descriptor
Portuguese
:
Epidermólise Bolhosa Juncional
Synonyms
English
:
Epidermolysis Bullosa Letalis
Herlitz Disease
Junctional Epidermolysis Bullosa
Lethal Junctional Epidermolysis Bullosa
Tree Number:
C16.131.831.493.170
C16.320.850.275.170
C17.800.804.493.170
C17.800.827.275.170
C17.800.865.410.170
Definition
English
:
Form of
epidermolysis bullosa
having onset at birth or during the neonatal period and transmitted through autosomal recessive inheritance. It is characterized by generalized
blister
formation, extensive
denudation
, and separation and cleavage of the basal cell
plasma
membranes
from the
basement membrane.
History Note
English
:
91; HERLITZ DISEASE was HERLITZ'S DISEASE 1991-92
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:
28926
Unique Identifier:
D016109
Occurrence in VHL
:
Similar:
DeCS
CID-10
SciELO
LILACS
LIS