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[Long-term survival in malignant atrophic papulosis: a case report and review of the Japanese literature].
[malignant atrophic papulosis]
Malignant
atrophic
papulosis
(
MAP
)
is
characterized
by
skin
lesion
and
high
mortality
rate
caused
by
perforation
of
the
GI
tract
or
involvement
of
the
central
nervous
system
.
Approximately
100
cases
have
been
reported
in
the
world
literatures
,
but
etiology
is
unknown
.
A
44
-
year
-old
female
patient
was
admitted
for
chest
and
abdominal
pain
with
characteristic
papuloses
,
which
had
been
noted
by
the
patient
seven
years
ago
.
Because
of
positive
peritoneal
irritation
and
intraabdominal
free
air
,
exploratory
laparotomy
was
done
.
Multiple
red
inflammatory
or
yellow
atrophic
maculae
on
the
entire
intestine
with
no
obvious
perforation
but
with
air-leak
were
found
.
Those
perforations
were
closed
with
seromuscular
sutures
.
The
patient
is
doing
well
sixteen
months
after
surgery
.
Fourteen
MAP
cases
have
been
reported
in
the
Japanese
literature
.
As
is
found
in
the
world
literature
,
the
mortality
rate
is
extremely
high
.
All
of
reported
cases
were
initially
diagnosed
because
of
the
particular
skin
lesions
.
Abdominal
symptoms
developed
in
10
cases
and
six
of
these
died
.
Three
cases
died
within
three
weeks
after
bowel
resection
.
There
are
three
surviving
cases
.
One
was
treated
conservatively
even
though
intraabdominal
free
air
was
present
.
Two
had
three
operations
,
including
one
simple
closure
and
two
intraabdominal
explorations
.
According
to
this
result
,
a
bowel
resection
should
not
be
performed
on
MAP
patients
because
of
this
high
mortality
.
Administration
of
anticoagulants
,
i
.
e
.
,
heparin
,
prostaglandin
E
1
and
ticlopidine
seems
to
be
effective
in
alleviating
symptoms
and
might
prevent
further
deterioration
.
Diseases
Validation
Diseases presenting
"high mortality rate caused by perforation of the gi tract or involvement of the central nervous system"
symptom
malignant atrophic papulosis
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