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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
"behavioral deterioration"
symptom
homocystinuria without methylmalonic aciduria
x-linked adrenoleukodystrophy
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