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Malignant atrophic papulosis.
[malignant atrophic papulosis]
Malignant
atrophic
papulosis
(
MAP
;
also
known
as
Degos
'
disease
)
has
a
purely
cutaneous
variant
and
a
systemic
variant
with
cutaneous
manifestations
.
Both
have
similar
cutaneous
eruptions
.
MAP
manifests
as
erythematous
,
pink
or
red
papules
(
2
-
15
mm
)
,
which
evolve
into
scars
with
central
,
porcelain-
white
atrophic
centres
.
Purely
cutaneous
MAP
is
a
benign
condition
that
can
be
life-
long
.
Systemic
MAP
has
a
grim
prognosis
,
but
is
not
uniformly
fatal
.
The
cause
of
death
is
usually
intestinal
perforation
.
Death
usually
occurs
within
2
-
3
years
from
the
onset
of
systemic
involvement
.
Systemic
MAP
can
involve
the
nervous
,
opthalmological
,
gastrointestinal
,
cardiothoracic
and
hepatorenal
systems
.
No
specific
laboratory
test
can
be
used
to
aid
in
diagnosing
MAP
.
Histopathologically
,
a
wedge-shaped
degeneration
of
collagen
is
present
with
a
prominent
interface
reaction
with
squamatization
of
the
dermoepidermal
junction
,
melanin
incontinence
and
epidermal
atrophy
.
No
treatment
has
been
shown
to
be
effective
in
the
treatment
of
MAP
.