Lifelong urticaria and multiple unexplained systemic symptoms

Abstract

Conflict of interest: none declared. A 23‐year‐old man had a history of a recurrent, urticated, mildly itchy eruption since infancy, of myalgia and headaches since childhood, and an episode of aseptic meningitis at 14 years of age. Other symptoms included mouth and genital ulceration, sensorineural deafness, and conjunctivitis. Sudden onset of visual blurring led to admission for neurological investigation. On physical examination, an urticarial eruption was seen on the trunk and arms (Fig. 1), with mild muscle weakness and bilateral papillo‐oedema (Fig. 1). Erythrocyte sedimentation rate and C‐reactive protein were raised (50 mm/h and 47 mg/L; normal ranges 0–15 mm/h and < 10 mg/L, respectively). Cerebrospinal fluid (CSF) examination was abnormal, with a raised white cell count (41 × 106/L and 60% polymorphs; normal 0–5 × 106/L and no neutrophils), raised protein (0.7 g/L; normal 0.2–0.4 g/L) and increased CSF opening pressure. Nerve conduction studies, electromyography, and brain magnetic resonance imaging gave normal results.