Idiopathic intracranial hypertension as the initial presentation of systemic lupus erythematosus.

A 14-year-old girl was referred for evaluation of headache with episodes of transient blurring of vision, and intermittent fever for 4 weeks. On examination she was conscious and febrile, with multiple annular purpuric skin lesions present over the face and back. Neurological examination revealed a bilaterally extensor plantar response, with bilateral papilloedema. Lumbar puncture yielded clear spinal fluid with a very high opening pressure with a normal biochemistry and cytology. Neuroimaging showed evidence of raised intracranial tension. She was provisionally diagnosed to have idiopathic intracranial hypertension (IIH) and started on anticerebral oedema measures. Despite medication, she continued to be symptomatic. On the sixth day of admission, her antinuclear antibody and antidouble-stranded DNA registered positively in high titres. She was diagnosed with systemic lupus erythematosus (SLE) with IIH and was started on corticosteroids, with dramatic recovery of her symptoms and clinical signs. Reports of SLE, the maiden presentation of which is IIH, are rare in the literature.