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[Malignant atrophic papulosis (Degos' syndrome) in a suckling infant].
[malignant atrophic papulosis]
The
clinical
and
histological
diagnosis
of
Degos
'
syndrome
could
be
made
in
a
8
-
month
-old
infant
with
scarring
skin
lesions
.
Some
weeks
later
the
baby
developed
severe
intestinal
symptoms
and
died
from
perforations
of
multiple
necrotic
lesions
of
the
small
bowel
.
Autopsy
revealed
in
addition
to
the
skin
and
gut
lesions
a
haemorrhagic
cortical
infarction
of
the
brain
and
fibrotic
areas
in
the
myocardium
.
In
all
these
lesions
characteristic
stenotic
endovascular
proliferations
were
seen
.
Malignant
atrophic
papulosis
is
an
extremely
rare
disease
in
infancy
(
our
patient
is
the
fifth
reported
case
)
with
a
well
defined
clinical
picture
,
characteristic
histological
appearance
of
obliterating
vasculitis
,
but
unknown
aetiology
.
Diseases
Validation
Diseases presenting
"fibrotic areas in the myocardium"
symptom
malignant atrophic papulosis
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