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[Malignant atrophic papulosis (Köhlmeier-Degos disease)].
[malignant atrophic papulosis]
On
the
basis
of
106
patients
with
malignant
atrophic
papulosis
in
the
literature
,
including
one
case
of
our
own
,
the
clinical
,
histopathological
and
nosological
features
of
this
disease
are
reviewed
.
Histopathologically
,
the
typical
papules
with
central
porcelain-
white
atrophy
show
broad
wedge-shaped
necrosis
of
the
connective
tissue
due
to
thrombotic
occlusions
of
small
vessels
in
the
corium
.
The
disease
leads
to
death
in
50
%
of
these
cases
,
mostly
due
to
involvement
of
the
gut
and
the
central
nervous
system
.
Malignant
atrophic
papulosis
is
the
prototype
of
a
disease
,
in
which
skin
lesions
are
the
primary
sign
of
a
systemic
disorder
demanding
the
interdisciplinary
cooperation
of
various
specialists
.
Diseases
Validation
Diseases presenting
"atrophy show"
symptom
malignant atrophic papulosis
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