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Possible involvement of SDF-1/CXCL12 in the pathogenesis of Degos disease.
[malignant atrophic papulosis]
Degos
disease
or
malignant
atrophic
papulosis
is
a
rare
occlusive
vasculopathic
disease
characterized
by
pathognomonic
cutaneous
lesions
and
frequently
fatal
systemic
involvement
.
The
etiology
of
malignant
atrophic
papulosis
remains
unclear
,
and
there
is
currently
no
effective
treatment
for
malignant
atrophic
papulosis
.
Several
chemokines
can
potentiate
and
expand
the
platelet
response
to
increase
thrombus
formation
.
Among
these
chemokines
,
this
study
examined
the
expression
of
stromal
cell-derived
factor
(
SDF
)
-
1
/
CXCL
12
,
which
is
secreted
by
bone
-marrow
stromal
and
endothelial
cells
,
activates
megakaryocyte
precursors
,
and
costimulates
platelet
activation
.
We
sought
to
investigate
and
compare
the
expression
of
SDF-
1
/
CXCL
12
in
tissue
sections
taken
from
2
patients
with
Degos
disease
,
2
patients
with
other
vaso-occlusive
diseases
,
and
2
healthy
control
subjects
.
Immunohistochemical
staining
involving
antibodies
to
SDF-
1
/
CXCL
12
was
performed
on
3
skin
biopsy
specimens
taken
from
2
patients
with
Degos
disease
,
1
from
a
patient
with
antiphospholipid
syndrome
,
1
from
a
patient
with
cryoglobulinemia
,
and
2
from
healthy
control
subjects
.
Strong
SDF-
1
/
CXCL
12
staining
was
observed
in
the
infiltrating
inflammatory
cells
in
the
perivascular
,
intravascular
,
and
perineural
areas
in
tissue
samples
from
patients
with
Degos
disease
.
No
staining
was
observed
in
samples
from
patients
with
antiphospholipid
syndrome
or
cryoglobulinemia
or
from
healthy
control
subjects
.
The
number
of
cases
available
for
evaluation
was
small
.
The
findings
were
based
primarily
on
the
immunohistochemical
results
and
were
not
confirmed
using
other
techniques
.
The
intense
staining
of
SDF-
1
/
CXCL
12
in
lesions
attributed
to
Degos
disease
,
demonstrated
for
the
first
time
to
our
knowledge
in
this
study
,
suggests
SDF-
1
/
CXCL
12
involvement
in
the
pathogenesis
of
the
disease
.
Diseases
Validation
Diseases presenting
"from healthy control subjects"
symptom
malignant atrophic papulosis
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