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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
"female patient"
symptom
achondroplasia
adrenal incidentaloma
alexander disease
aniridia
aromatase deficiency
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
coats disease
cohen syndrome
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentinogenesis imperfecta
epidermolysis bullosa simplex
esophageal squamous cell carcinoma
fabry disease
holt-oram syndrome
hydrocephalus with stenosis of the aqueduct of sylvius
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
pyruvate dehydrogenase deficiency
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
x-linked adrenoleukodystrophy
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