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The mystery of Sneddon syndrome: relationship with antiphospholipid syndrome and systemic lupus erythematosus.
[sneddon syndrome]
Since
its
description
in
1965
,
Sneddon
syndrome
(
SNS
)
is
usually
characterized
by
the
association
of
an
ischemic
cerebrovascular
disease
and
a
widespread
livedo
reticularis
.
The
presence
of
many
other
manifestations
suggests
that
it
is
a
systemic
syndrome
.
The
prevalence
of
anti-phospholipid
antibodies
(
aPL
)
is
highly
variable
,
41
%
in
our
experience
.
Comparison
of
patients
with
or
without
aPL
showed
that
the
fishnet
of
the
livedo
was
clearly
larger
in
aPL-negative
patients
who
nevertheless
,
did
not
develop
thrombocytopenia
.
Seizures
and
clinically
audible
mitral
regurgitation
were
more
frequently
observed
in
aPL-
positive
patients
.
These
data
lead
to
consider
that
SNS
is
not
a
unique
entity
.
As
patients
with
primary
anti-phospholipid
syndrome
(
APS
)
and
SNS
did
not
differ
from
those
with
livedo
reticularis
,
ischemic
cerebral
events
and
APS
within
systemic
lupus
erythematosus
(
SLE
)
,
there
is
no
reason
today
to
exclude
patients
with
SLE
.
On
one
hand
,
SNS
might
cover
a
continuum
spectrum
joining
diverse
clinico-biological
entities
ranging
from
aPL-negative
to
SLE-related
cases
,
with
primary
APS-SNS
standing
amidst
.
On
the
other
hand
,
one
might
speculate
that
SNS
should
be
regarded
as
a
nearly
similar
clinical
expression
of
two
distinct
disorders
,
i
.
e
.
a
peculiar
form
of
APS
characterized
by
preferential
arteriolar
involvement
or
on
the
opposite
a
primary
non-a
PL
related
small
artery
disease
mainly
involving
brain
and
skin
vessels
.
Diseases
Validation
Diseases presenting
"diverse clinico-biological entities"
symptom
sneddon syndrome
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