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[Köhlmeier-Degos disease (Malignant Atrophic Papulosis): a cause of recurrent multiple intestinal perforations].
[malignant atrophic papulosis]
Malignant
Atrophic
Papulosis
(
MAP
)
or
Degos
disease
is
a
very
rare
vasculopathy
affecting
multiple
systems
.
When
associated
with
gastrointestinal
tract
or
central
nervous
system
involvment
,
patients
with
Degos
disease
have
a
poor
prognosis
and
a
high
mortality
.
We
report
a
case
of
Degos
disease
with
systemic
involvement
,
which
ultimately
caused
peritonitis
,
sepsis
,
and
death
,
despite
all
treatment
.
A
29
-
year
-old
man
was
admitted
in
June
2007
on
the
surgical
ward
of
our
hospital
with
acute
generalized
abdominal
pain
and
multiple
skin
lesions
.
The
patient
had
an
appendectomy
at
another
hospital
6
weeks
before
the
current
presentation
.
The
exploratory
laparotomy
showed
2
perforations
of
the
ileum
.
After
the
initial
procedure
,
the
patient
developed
spontaneous
recurrent
perforations
of
the
small
bowel
and
suffered
another
3
reoperations
.
Neuroradiologic
reports
described
central
and
peripheral
nervous
system
involvement
with
progressive
clinical
deterioration
and
a
meningovascular
pattern
at
cerebral
MRI
.
Despite
aggressive
treatment
the
patient
died
3
months
after
the
first
surgical
intervention
.
Diseases
Validation
Diseases presenting
"abdominal pain"
symptom
22q11.2 deletion syndrome
adrenal incidentaloma
alpha-thalassemia
benign recurrent intrahepatic cholestasis
carcinoma of the gallbladder
child syndrome
cholangiocarcinoma
congenital diaphragmatic hernia
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
erdheim-chester disease
erythropoietic protoporphyria
fabry disease
familial hypocalciuric hypercalcemia
familial mediterranean fever
focal myositis
liposarcoma
lymphangioleiomyomatosis
malignant atrophic papulosis
pleomorphic liposarcoma
primary effusion lymphoma
primary hyperoxaluria type 1
proteus syndrome
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
typhoid
von hippel-lindau disease
well-differentiated liposarcoma
This symptom has already been validated