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Atypical presentation of neuropsychiatric lupus with acanthosis nigricans.
[x-linked adrenoleukodystrophy]
Cutaneous
manifestations
are
commonly
observed
in
pediatric
patients
with
systemic
lupus
erythematosus
.
Acanthosis
nigricans
,
however
,
is
rarely
reported
,
and
even
less
often
in
association
with
neuropsychiatric
manifestations
of
lupus
erythematosus
.
We
describe
a
9
-
year
-old
boy
with
acute
behavioral
and
cognitive
deterioration
,
combined
with
cutaneous
,
diffuse
hyperpigmented
and
hyperkeratotic
plaques
.
Cerebral
magnetic
resonance
imaging
revealed
cortical
atrophy
and
white
matter
abnormalities
involving
the
fornix
,
corpus
callosum
,
and
parieto-
occipital
periventricular
regions
.
The
presence
of
progressive
cognitive
and
behavioral
deterioration
,
combined
with
abnormal
white
matter
signals
on
magnetic
resonance
imaging
,
led
us
to
suspect
X-
linked
adrenoleukodystrophy
.
The
subsequent
development
of
systemic
signs
,
together
with
positive
serologic
tests
,
confirmed
the
diagnosis
of
neuropsychiatric
lupus
with
acanthosis
nigricans
.
We
review
the
literature
on
acanthosis
nigricans
in
systemic
lupus
erythematosus
and
the
value
of
magnetic
resonance
imaging
in
evaluating
patients
with
neuropsychiatric
systemic
lupus
.
Diseases
Validation
Diseases presenting
"corpus callosum"
symptom
adrenomyeloneuropathy
alexander disease
cadasil
canavan disease
classical phenylketonuria
cohen syndrome
gm1 gangliosidosis
hirschsprung disease
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
kallmann syndrome
krabbe disease
monosomy 21
neonatal adrenoleukodystrophy
oligodontia
proteus syndrome
pyruvate dehydrogenase deficiency
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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