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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
"dementia"
symptom
adrenomyeloneuropathy
alexander disease
cadasil
canavan disease
carcinoma of the gallbladder
cushing syndrome
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
inclusion body myositis
phenylketonuria
sneddon syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated