Rare Diseases Symptoms Automatic Extraction
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Case for diagnosis.
[malignant atrophic papulosis]
Degos
disease
,
also
known
as
malignant
atrophic
papulosis
,
is
a
rare
occlusive
vasculopathy
of
unknown
etiology
characterized
by
infarcts
in
the
dermis
,
gastrointestinal
tract
,
central
nervous
system
,
and
other
organs
.
It
is
characterized
by
papules
,
which
become
umbilicated
and
evolve
with
a
depressed
porcelain-
white
central
area
,
with
an
erythematous
halo
with
telangiectasias
.
Histological
findings
include
wedge-shaped
dermoepidermal
necrosis
and
blood
vessel
thrombosis
.
Approximately
50
-
60
%
of
patients
with
systemic
symptoms
die
within
2
-
3
years
,
most
due
to
gastrointestinal
perforation
.
We
report
a
typical
case
,
with
lethal
outcome
,
in
a
45
-
year
-old
woman
.
Diseases
Validation
Diseases presenting
"porcelain-white central area"
symptom
malignant atrophic papulosis
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