Rare Diseases Symptoms Automatic Extraction
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[Malignant atrophic papulosis (Degos' syndrome)].
[malignant atrophic papulosis]
In
a
49
-
year
-old
female
patient
with
an
eruption
of
lentil-shaped
papules
on
the
trunk
and
extremities
,
persisting
for
six
years
,
originally
in
another
department
scleroderma
guttata
was
diagnosed
.
For
one
year
the
patient
suffered
also
from
dyspeptic
complaints
,
loss
of
weight
and
relapsing
polyserositis
.
The
complaints
receded
temporarily
after
immunosuppressive
therapy
.
On
surgical
revision
of
the
abdominal
cavity
on
account
of
serous
peritonitis
during
the
last
three
months
eruption
of
whitish
foci
on
the
intestinal
serosa
and
omentum
was
found
.
Bioptic
excision
of
the
skin
revealed
an
old
bland
infarct
.
From
the
clinical
course
and
dermatological
picture
papulosis
maligna
atrophicans
(
Degos
'
syndrome
)
was
diagnosed
.
Autoptic
examination
confirmed
the
clinical
diagnosis
of
the
syndrome
by
the
finding
of
thromangiitis
of
Bürger
's
type
with
bland
infarcts
of
the
small
intestine
and
perforation
of
the
jejunum
.
Diseases
Validation
Diseases presenting
"scleroderma"
symptom
focal myositis
kindler syndrome
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
oral submucous fibrosis
systemic capillary leak syndrome
werner syndrome
This symptom has already been validated
