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Degos' disease with delayed involvement of the gastrointestinal tract.
[malignant atrophic papulosis]
A
75
-
year
-old
woman
presented
with
pink
papules
over
the
trunk
and
limbs
.
Over
the
next
few
weeks
,
the
papules
became
umbilicated
and
then
developed
into
porcelain-
white
lesions
with
a
rose
or
violaceous
rim
.
A
biopsy
of
one
of
these
lesions
revealed
superficial
and
deep
dermal
lymphohistiocytic
inflammation
,
mainly
perivascular
with
a
central
zone
of
infarction
consistent
with
Degos
'
disease
.
Three
years
later
,
she
presented
with
increasing
abdominal
pain
,
anaemia
,
raised
inflammatory
markers
and
an
abdominal
bruit
.
Angiography
showed
splanchnic
vessel
stenosis
possibly
secondary
to
vasculitis
and
she
underwent
successful
balloon
dilatation
.
Treatment
with
prednisolone
was
commenced
.
There
has
been
no
recurrence
of
lesions
in
the
12
months
since
the
balloon
dilatation
.
Diseases
Validation
Diseases presenting
"inflammatory markers"
symptom
acute rheumatic fever
dystrophic epidermolysis bullosa
erdheim-chester disease
inclusion body myositis
krabbe disease
legionellosis
malignant atrophic papulosis
pyomyositis
scrub typhus
sneddon syndrome
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