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[Sneddon syndrome: vasculitis or thrombotic disorder?].
[sneddon syndrome]
Livedo
reticularis
generalisata
(
LR
)
in
combination
with
affection
of
CNS
is
referred
to
as
Sneddon
's
syndrome
(
SNS
)
.
Latest
data
suggest
chronic
progressive
systemic
disorder
with
occlusion
of
small
and
medium
sized
vessels
(
e
.
g
.
,
cutis
,
brain
,
kidneys
,
heart
,
eyes
)
.
No
conclusive
etiology
is
known
,
though
there
are
correlations
to
the
antiphospholipid
syndrome
,
systemic
secondary
vasculitis
and
coagulopathies
.
Hereditary
and
toxic
factors
seem
to
play
a
role
in
pathogenesis
in
some
cases
.
Diagnostic
procedure
and
clinical
course
of
a
56
-
year
-old
woman
with
dementia
and
hemiparesis
proceeded
by
LR
is
reported
.
MRI-
,
SPECT-
and
TCD-findings
were
congruent
with
diffuse
ischemic
lesions
of
the
brain
due
to
affection
of
small
-
and
medium-sized
vessels
.
Histopathological
specimens
of
the
brain
,
meninges
and
cutis
were
non
diagnostic
.
Some
laboratory
findings
suggested
vasculitis
as
an
underlying
cause
.
LR
improved
under
immunosuppressive
therapy
with
prednisolone
and
azathioprin
.
SNS
does
not
seem
to
be
a
nosological
entity
.
A
differentiation
between
primary
(
idiopathic
)
and
secondary
SNS
is
useful
for
different
therapeutical
approaches
.