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Junctional epidermolysis bullosa in a female Nigerian child: a case report.
[junctional epidermolysis bullosa]
To
report
the
case
of
Junctional
Epidermolysis
bullosa
seen
in
the
University
of
Calabar
Teaching
Hospital
,
Calabar
and
to
draw
attention
to
the
existence
of
this
condition
among
Nigerians
.
A
Nigerian
female
infant
,
normal
at
birth
,
developed
extensive
blistering
skin
eruptions
from
the
age
of
ten
days
.
The
lesions
involved
the
face
,
neck
,
shoulders
,
upper
trunk
and
the
buccal
mucosa
of
the
lower
lip
sparing
the
limbs
.
The
cornea
were
cloudy
.
The
history
,
type
and
distribution
of
the
lesions
were
consistent
with
Junctional
Epidermolysis
bullosa
(
EB
)
.
This
was
confirmed
by
skin
biopsy
and
histology
.
Various
medications
including
systemic
and
topical
steroids
,
antibiotics
and
other
topical
creams
only
produce
transient
relief
with
frequent
relapses
.
The
Junctional
EB
type
is
known
to
be
autosomal
recessive
in
inheritance
though
there
was
no
positive
family
history
.
Avoidance
of
heat
,
warm
bath
and
trauma
and
genetic
counseling
can
be
helpful
in
reducing
frequent
relapses
of
the
condition
that
has
no
specific
treatment
.
This
appears
to
be
the
first
case
of
Epidermolysis
bullosa
to
be
reported
from
Calabar
,
south
eastern
Nigeria
.
This
case
report
shows
that
this
rare
condition
does
affect
people
in
the
tropics
.
Efforts
should
always
be
made
to
reach
a
definitive
diagnosis
whenever
unusual
conditions
are
encountered
in
clinical
practice
.
Diseases
Validation
Diseases presenting
"other topical creams"
symptom
junctional epidermolysis bullosa
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