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Painful penile ulceration in a patient with malignant atrophic papulosis.
[malignant atrophic papulosis]
Malignant
atrophic
papulosis
(
MAP
)
is
a
rare
,
obliterating
vasculopathy
affecting
multiple
systems
,
frequently
with
a
poor
prognosis
.
Although
cutaneous
lesions
are
often
the
initial
presentation
,
systemic
involvement
is
also
common
,
usually
with
a
fatal
outcome
.
Involvement
of
the
genitalia
is
very
rare
.
We
describe
a
45
-
year
-old
male
patient
with
multisytemic
manifestation
of
MAP
accompanied
by
painful
penile
ulceration
.
The
pathogenesis
of
MAP
is
not
yet
fully
understood
and
effective
treatment
choices
are
limited
.
In
our
case
,
the
combination
of
pentoxifylline
and
dipyridamole
failed
to
provide
a
beneficial
effect
on
the
progression
of
the
disease
and
the
patient
died
due
to
intestinal
and
intrathoracic
manifestation
of
MAP
.
In
the
present
case
,
attention
should
be
drawn
to
the
following
clinical
course
and
therapeutic
properties
:
(
i
)
we
describe
the
second
patient
in
the
literature
diagnosed
with
MAP
and
painful
penile
ulceration
;
(
ii
)
to
our
knowledge
,
this
is
the
first
reported
case
with
oesophageal
fistula
due
to
MAP
;
(
iii
)
we
could
not
confirm
the
efficacy
of
pentoxifylline
,
the
recently
reported
treatment
modality
,
in
our
patient
.
Diseases
Validation
Diseases presenting
"first reported case"
symptom
achondroplasia
acute rheumatic fever
alexander disease
allergic bronchopulmonary aspergillosis
aniridia
cutaneous mastocytosis
dedifferentiated liposarcoma
epidermolysis bullosa simplex
fabry disease
focal myositis
harlequin ichthyosis
heparin-induced thrombocytopenia
kabuki syndrome
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
pleomorphic liposarcoma
primary hyperoxaluria type 1
thoracic outlet syndrome
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wolf-hirschhorn syndrome
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