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Degos' syndrome complicated by bowel perforation: focus on radiological findings.
[malignant atrophic papulosis]
We
describe
a
50
-
year
-old
man
who
first
presented
with
multiple
skin
lesions
which
were
characteristic
of
Degos
'
syndrome
.
The
patient
developed
multiple
episodes
of
abdominal
pain
.
Some
episodes
resolved
with
conservative
management
,
for
others
he
underwent
urgent
operations
for
bowel
perforations
.
The
patient
subsequently
underwent
extensive
small
bowel
resection
,
but
further
systemic
deterioration
ensued
and
he
died
.
The
imaging
findings
of
Degos
'
syndrome
and
the
implications
of
pneumatosis
intestinalis
and
pneumoperitoneum
are
discussed
.
Diseases
Validation
Diseases presenting
"skin lesions"
symptom
child syndrome
cowden syndrome
cutaneous mastocytosis
cystinuria
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
familial mediterranean fever
focal myositis
gm1 gangliosidosis
heparin-induced thrombocytopenia
hirschsprung disease
junctional epidermolysis bullosa
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
oligodontia
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
proteus syndrome
severe combined immunodeficiency
sneddon syndrome
waldenström macroglobulinemia
werner syndrome
wiskott-aldrich syndrome
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