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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
"first time"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
gm1 gangliosidosis
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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