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Extensive acantholysis as the major histological feature of a severe case of Dowling Meara-epidermolysis bullosa simplex: a reappraisal of acantholysis in the newborn.
[epidermolysis bullosa simplex]
Epidermolysis
bullosa
(
EB
)
is
a
heterogeneous
group
of
inherited
skin
disorders
characterized
by
blistering
and
skin
fragility
secondary
to
mechanical
trauma
.
Epidermolysis
bullosa
simplex
(
EBS
)
is
the
most
frequent
form
of
EB
,
with
Dowling-
Meara
(
DM
-
EBS
)
subtype
being
the
most
severe
form
in
this
group
.
Conventional
histopathological
evaluation
is
usually
of
low
value
in
the
diagnosis
of
EB
,
and
significant
histological
features
have
rarely
been
reported
in
this
group
of
diseases
.
We
describe
a
case
of
severe
DM
-
EBS
in
which
acantholysis
was
observed
in
the
histological
examination
.
This
finding
led
us
to
consider
other
diagnoses
,
such
as
neonatal
pemphigus
vulgaris
or
lethal
acantholytic
EB
.
Histological
,
immunological
,
ultrastructural
and
genetic
tests
were
performed
,
leading
to
a
final
diagnosis
of
DM
-
EBS
.
Therefore
,
we
believe
that
DM
-
EBS
should
be
considered
in
the
differential
diagnosis
of
a
newborn
with
blisters
,
where
acantholysis
is
the
main
histological
feature
.
