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Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients.
[sneddon syndrome]
Sneddon
syndrome
is
characterized
by
the
association
of
livedo
reticularis
and
cerebral
ischemic
arterial
events
(
stroke
or
transient
ischemic
attack
)
.
Reported
prevalence
of
antiphospholipid
antibodies
is
highly
variable
.
We
conducted
this
study
to
compare
the
clinical
and
pathologic
features
of
patients
with
Sneddon
syndrome
according
to
the
presence
or
absence
of
antiphospholipid
antibodies
.
Forty
-
six
consecutive
patients
with
Sneddon
syndrome
were
analyzed
.
All
were
examined
by
the
same
dermatologist
who
classified
the
livedo
of
the
trunk
according
to
the
regularity
of
the
fishnet
reticular
pattern
and
according
to
the
thickness
of
the
fishnet
reticular
pattern
(
>
or
=
10
mm
=
large
;
<
10
mm
=
fine
)
.
Skin
biopsies
were
systematically
performed
,
from
both
the
center
and
the
violaceous
netlike
pattern
in
38
patients
.
Antiphospholipid
antibodies
-
positive
Sneddon
syndrome
was
defined
by
the
presence
of
lupus
anticoagulant
or
abnormal
titers
of
anticardiolipin
antibodies
on
repeated
determinations
.
Group
I
consisted
of
27
antiphospholipid
antibodies
-negative
patients
and
Group
II
,
of
19
antiphospholipid
antibodies
-
positive
patients
.
All
patients
except
I
in
Group
II
had
irregular
livedo
reticularis
.
Large
livedo
racemosa
was
more
frequently
observed
in
Group
I
(
89
%
)
than
in
Group
II
(
21
%
,
p
<
0
.
001
)
.
On
skin
biopsy
,
arteriolar
obstruction
was
detected
in
only
8
patients
(
4
in
each
group
)
.
The
following
parameters
were
not
statistically
different
between
the
2
groups
:
gender
,
mean
age
at
detection
of
livedo
,
mean
age
at
first
clinical
cerebral
event
,
hypertension
,
Raynaud
phenomenon
,
patients
with
extracerebral
and
extracutaneous
arterial
or
arteriolar
thrombosis
or
stenosis
,
patients
with
venous
thrombosis
,
and
women
with
2
fetal
losses
or
more
.
In
contrast
,
seizures
(
11
%
in
Group
I
versus
37
%
in
Group
II
,
p
<
0
.
05
)
,
mitral
regurgitation
on
echocardiogram
(
19
%
versus
53
%
,
p
=
0
.
02
)
,
and
thrombocytopenia
<
150
,
000
/
muL
(
0
%
versus
42
%
,
p
<
0
.
005
)
were
more
frequently
observed
in
Group
II
.
The
number
of
events
per
year
of
follow-up
was
lower
with
antiplatelet
therapy
(
0
.
08
versus
0
.
5
)
in
Group
I
,
but
was
not
different
with
anticoagulation
(
0
.
056
versus
0
.
06
)
.
Antiphospholipid
antibodies
-negative
and
-
positive
patients
with
Sneddon
syndrome
belong
to
close
but
different
subsets
of
Sneddon
syndrome
.
Diseases
Validation
Diseases presenting
"abnormal titers"
symptom
sneddon syndrome
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