[The 451(th) case: intermittent rash, fever and headache]

A 29-year-old woman was admitted to the Department of Rheumatology, Peking Union Medical College Hospital due to intermittent rashes, fever and headache. Palpable purpura were symmetrically distributed on the extremities and trunk. Other manifestations included headache with nausea and vomiting. Elevated white blood cell (WBC) count, platelet (PLT) count, erythrocyte sedimentation rate (ESR) and C-reactive protein were the main laboratory findings. Antinuclear antibodies and antineutrophil cytoplasmic antibodies were negative. Examination of the cerebrospinal fluid (CSF) revealed high intracranial pressure, while routine cytology and biochemical tests of CSF were normal. Head MRI scan and PET-CT did not detect remarkable findings. A diagnosis of systemic vasculitis was confirmed by the biopsy of skin lesion which showed inflammatory infiltration of the muscular vessel wall. Combination therapy of corticosteroids and cyclophosphamide lead to a rapid improvement in clinical symptoms and laboratory parameters. The patient was in stable remission till 6 month follow-up.