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Non-herlitz junctional epidermolysis bullosa.
[junctional epidermolysis bullosa]
Non-
Herlitz
junctional
epidermolysis
bullosa
(
nH
JEB
)
is
characterized
by
generalized
blisters
that
predominate
in
sites
exposed
to
friction
,
trauma
,
or
heat
.
Whereas
infants
and
children
with
nH
JEB
often
appear
to
resemble
patients
with
other
forms
of
EB
,
adults
with
this
disorder
typically
display
atrophic
scars
,
hypopigmentation
,
or
hyperpigmentation
at
sites
of
healed
blisters
as
well
as
incomplete
alopecia
,
dystrophic
nails
,
mucous
membrane
involvement
,
and
dental
abnormalities
.
Mild
(
or
severe
)
disease
early
in
life
may
be
characterized
by
the
opposite
phenotype
in
adults
with
nH
JEB
.
Although
nH
JEB
is
generally
less
severe
than
Herlitz
disease
,
fatalities
(
especially
in
neonates
)
are
not
uncommon
among
patients
with
the
former
diagnosis
.
Diseases
Validation
Diseases presenting
"incomplete alopecia"
symptom
junctional epidermolysis bullosa
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