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[Degos' disease as a cutaneous manifestation of systemic lupus erythematosus].
[malignant atrophic papulosis]
Degos
'
disease
or
malignant
atrophic
papulosis
is
a
rare
vasculopathy
characterized
by
the
presence
of
a
typical
skin
lesion
and
visceral
vascular
involvement
of
small
vessels
,
mainly
of
the
digestive
tract
or
central
nervous
system
.
The
most
interesting
fact
in
this
disease
is
the
benign
appearance
of
cutaneous
lesion
,
hiding
the
occlusion
of
skin
and
visceral
vessels
.
The
author
reports
the
case
of
a
female
patient
with
systemic
lupus
erythematosus
for
eight
years
.
During
her
follow
up
,
generalized
skin
papules
were
observed
on
the
trunk
and
limbs
,
sparing
her
face
,
hands
and
feet
,
compatible
with
Degos
'
disease
.
Additional
imaging
investigation
excluded
systemic
involvement
of
the
disease
.
Treatment
with
acetylsalicylic
acid
prevented
the
appearance
of
new
cutaneous
manifestations
and
the
patient
remains
clinically
stable
on
the
Outpatient
Clinic
without
complications
,
until
this
moment
.
Malign
atrophic
papulosis
is
a
rare
disease
with
a
poor
prognosis
.
However
,
its
association
with
systemic
lupus
erythematosus
seems
to
follow
a
more
benign
course
,
without
the
typical
visceral
involvement
.
Diseases
Validation
Diseases presenting
"central nervous system"
symptom
22q11.2 deletion syndrome
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alexander disease
aniridia
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hirschsprung disease
hodgkin lymphoma, classical
kabuki syndrome
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
legionellosis
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malignant atrophic papulosis
monosomy 21
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phenylketonuria
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zellweger syndrome
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