Rare Diseases Symptoms Automatic Extraction
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Lethal systemic Degos disease with prominent cardio-pulmonary involvement.
[malignant atrophic papulosis]
Degos
disease
DD
is
a
rare
obstructive
vasculopathy
characterized
by
distinctive
skin
lesions
.
Involvement
of
the
soles
,
palms
and
genitalia
is
rare
.
In
most
cases
,
disease
has
an
unfavorable
course
and
involves
gastrointestinal
tract
,
central
nervous
system
and
occasionally
other
organs
.
Pleural
and
pericardial
involvements
are
usually
minor
manifestations
with
prolonged
course
.
Death
occurs
in
approximately
50
%
of
the
patients
usually
due
to
intestinal
perforation
or
central
nervous
system
bleeding
.
We
describe
a
48
-
year
-old
man
of
lethal
systemic
DD
.
Widespread
skin
lesions
with
involvement
of
palmoplantar
surfaces
,
genitalia
and
scalp
were
ignored
for
3
years
,
whereas
the
disease
revealed
own
malignant
nature
.
The
disorder
progressed
to
nervous
,
gastrointestinal
and
cardiopulmonary
system
that
led
to
death
after
5
months
from
onset
of
systemic
involvement
as
severe
restrictive
cardio-
pulmonary
insufficiency
.
Autopsy
showed
diffuse
fibrotic
changes
in
serosal
membranes
and
internal
organs
.