Rare Diseases Symptoms Automatic Extraction
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Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis.
[malignant atrophic papulosis]
Malignant
atrophic
papulosis
(
MAP
)
,
or
Degos
syndrome
,
is
a
rare
disorder
of
unknown
etiology
.
It
is
characterized
by
a
deep
subcutaneous
vasculopathy
resulting
in
atrophic
,
porcelain-
white
papules
.
We
report
the
case
of
a
42
-
year
-old
woman
with
a
history
of
progressive
systemic
sclerosis
who
presented
with
painful
subcutaneous
nodules
on
her
abdomen
along
with
chronic
atrophic
papules
on
her
upper
and
lower
limbs
.
Biopsy
results
of
both
types
of
lesions
revealed
vascular
thrombi
without
surrounding
inflammation
.
We
briefly
review
the
literature
on
MAP
and
its
association
with
various
connective
tissue
diseases
.
To
our
knowledge
,
there
have
been
no
previous
reports
of
a
patient
with
the
clinical
and
histologic
presentations
described
here
.
Although
the
histologic
appearance
of
the
subcutaneous
nodules
was
very
similar
to
that
of
the
atrophic
papules
,
the
clinical
characteristics
of
the
2
types
of
lesions
were
strikingly
different
.
It
is
fair
to
theorize
that
Degos
lesions
do
not
start
as
atrophic
porcelain-
white
papules
but
rather
evolve
from
a
primary
lesion
.
We
hypothesize
that
these
lesions
start
as
painful
red
nodules
and
may
represent
part
of
the
disease
spectrum
in
the
evolution
of
MAP
.
