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Malignant and benign forms of atrophic papulosis (Köhlmeier-Degos disease): systemic involvement determines the prognosis.
[malignant atrophic papulosis]
Atrophic
papulosis
(
Köhlmeier-
Degos
disease
)
is
a
rare
disease
of
unknown
aetiology
.
The
cutaneous
signs--papular
skin
lesions
with
central
porcelain-
white
atrophy
and
surrounding
telangiectatic
rim--are
almost
pathognomonic
.
Extracutaneous
,
systemic
involvement
includes
multiple
limited
infarcts
of
the
gastrointestinal
system
,
central
nervous
system
and
other
organs
.
To
assess
prospectively
the
demographics
,
epidemiological
data
and
prognosis
of
patients
with
atrophic
papulosis
evaluated
in
a
single
centre
.
A
prospective
,
single
-centre
,
cohort
study
at
diagnosis
was
performed
on
a
series
of
39
patients
with
atrophic
papulosis
,
first
seen
between
2000
and
2007
and
evaluated
up
to
2012
.
The
occurrence
of
cutaneous
lesions
defined
the
onset
of
disease
in
all
cases
.
The
mean
age
of
onset
was
35
.
4
±
12
.
3
years
and
the
male-
to
-
female
ratio
was
1
:
1
.
4
.
In
total
,
9
%
of
patients
reported
familial
occurrence
.
Extracutaneous
(
systemic
)
signs
were
recorded
in
29
%
of
the
patients
,
whereas
the
median
time
for
development
of
systemic
manifestations
was
1
year
(
0
.
03
-
0
.
97
quantiles
:
0
-
7
years
)
after
the
occurrence
of
cutaneous
lesions
.
The
prognosis
was
determined
mainly
by
the
presence
of
systemic
involvement
.
73
%
of
the
patients
with
systemic
manifestations
(
73
%
developed
intestinal
perforation
)
died
,
while
none
of
the
patients
with
only
cutaneous
disease
had
a
lethal
outcome
.
The
cumulative
5
-
year
survival
rate
in
patients
with
systemic
disease
was
54
.
5
%
.
Atrophic
papulosis
,
previously
called
malignant
atrophic
papulosis
,
should
be
classified
into
a
malignant
,
systemic
form
and
a
benign
,
cutaneous
one
,
the
latter
being
more
common
.
The
probability
of
having
a
benign
form
of
the
disease
at
onset
is
approximately
70
%
,
increasing
to
97
%
after
7
years
of
monosymptomatic
cutaneous
course
.
Diseases
Validation
Diseases presenting
"only cutaneous disease"
symptom
malignant atrophic papulosis
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