Rare Diseases Symptoms Automatic Extraction

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 presenting "systemic lupus erythematosus" symptom

  • acute rheumatic fever
  • familial mediterranean fever
  • focal myositis
  • hodgkin lymphoma, classical
  • inclusion body myositis
  • malignant atrophic papulosis
  • oligodontia
  • papillon-lefèvre syndrome
  • primary hyperoxaluria type 1
  • pyomyositis
  • sneddon syndrome
  • werner syndrome
  • x-linked adrenoleukodystrophy

This symptom has already been validated