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Dystrophic epidermolysis bullosa in pregnancy: a case report of the autosomal dominant subtype and review of the literature.
[dystrophic epidermolysis bullosa]
Epidermolysis
bullosa
(
EB
)
is
a
group
of
inherited
blistering
skin
diseases
that
vary
widely
in
their
pathogenesis
and
severity
.
There
are
three
main
categories
of
EB
:
simplex
,
junctional
,
and
dystrophic
.
This
classification
is
based
on
the
level
of
tissue
separation
within
the
basement
membrane
zone
and
this
is
attributed
to
abnormalities
of
individual
or
several
anchoring
proteins
that
form
the
interlocking
network
spanning
from
the
epidermis
to
the
dermis
underneath
.
Dystrophic
EB
results
from
mutations
in
COL
7
A
1
gene
coding
for
type
VII
collagen
leading
to
blister
formation
within
the
dermis
.
Diagnosis
ultimately
depends
on
the
patient
's
specific
genetic
mutation
,
but
initial
diagnosis
can
be
made
from
careful
examination
and
history
taking
.
We
present
a
pregnant
patient
known
to
have
autosomal
dominant
dystrophic
EB
and
discuss
the
obstetrical
and
neonatal
outcome
.
The
paper
also
reviews
the
current
English
literature
on
this
rare
skin
disorder
.