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Livedo, dementia, thrombocytopenia, and endotheliitis without antiphospholipid antibodies: seronegative antiphospholipid-like syndrome.
[sneddon syndrome]
We
report
a
51
-
year
-old
woman
who
presented
with
dementia
,
livedo
racemosa
,
polyarthralgia
,
mild
renal
insufficiency
,
proteinuria
,
and
thrombocytopenia
.
Cutaneous
and
renal
biopsy
specimens
both
showed
an
identical
specific
occlusive
arteriolopathy
consistent
with
Sneddon
syndrome
and
antiphospholipid
syndrome
.
However
,
no
antiphospholipid
antibodies
were
detected
and
we
,
therefore
,
diagnosed
seronegative
antiphospholipid-like
syndrome
.
We
discuss
the
nosology
of
this
entity
and
its
association
with
non-
antiphospholipid
antibody
-related
Sneddon
syndrome
.
The
common
denominator
of
Sneddon
syndrome
and
antiphospholipid
syndrome
with
or
without
antiphospholipid
antibodies
seems
to
be
the
endothelial
damage
and
occlusive
arteriolopathy
.
Skin
biopsy
is
useful
to
confirm
the
diagnosis
of
seronegative
antiphospholipid-like
syndrome
.
Diseases
Validation
Diseases presenting
"renal biopsy specimens"
symptom
sneddon syndrome
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