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Malignant melanoma arising in the setting of epidermolysis bullosa simplex: an important distinction from epidermolysis bullosa nevus.
[epidermolysis bullosa simplex]
Patients
with
epidermolysis
bullosa
(
EB
)
do
not
carry
a
significantly
increased
risk
of
melanoma
but
are
prone
to
developing
large
,
markedly
atypical
melanocytic
nevi
(
EB
nevi
)
,
which
may
mimic
melanoma
clinically
and
histologically
.
Many
authors
now
favor
a
conservative
approach
in
managing
atypical
pigmented
lesions
in
patients
with
EB
.
We
present
the
case
of
a
30
-
year
-old
woman
with
severe
EB
simplex
who
sought
care
for
a
large
red
and
black
ulcerated
plaque
.
The
clinical
differential
diagnosis
included
EB
nevus
and
melanoma
.
An
incisional
punch
biopsy
specimen
revealed
an
atypical
melanocytic
proliferation
with
focal
florid
pagetoid
spread
and
involving
elongated
rete
ridges
,
consistent
with
invasive
acral
lentiginous
melanoma
.
The
subsequent
amputation
was
confirmatory
.
Micrometastasis
was
detected
in
1
of
5
sentinel
lymph
nodes
.
To
our
knowledge
,
this
is
the
first
reported
case
of
melanoma
arising
in
EB
simplex-affected
skin
.
It
highlights
the
difficulty
in
differentiating
melanoma
from
an
EB
nevus
.
Despite
the
increasing
awareness
of
EB
nevi
,
a
high
index
of
suspicion
for
melanoma
should
be
maintained
,
and
early
biopsy
is
recommended
when
evaluating
large
pigmented
lesions
in
patients
with
EB
.
Diseases
Validation
Diseases presenting
"focal florid pagetoid"
symptom
epidermolysis bullosa simplex
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