Case Studies

 Case 10.6 Behçet's syndrome

A 32-year-old man from a Turkish family presented with deteriorating vision and painful swollen knees. Further questioning revealed a 10-year history of relapsing and remitting mouth ulcers and a less severe history of genital ulceration. On examination he had reduced visual acuity associated with a florid retinal vasculitis. Two 1-cm mouth ulcers were found but no active genital ulceration. He had synovitis in both knees. Investigation revealed a raised erythrocyte sedimentation rate at 94mm/h but a normal blood count and negative tests for rheumatoid factor, antinuclear antibodies, cytomegalovirus and HIV infection. A clinical diagnosis of Behcet's syndrome was made. He was treated with high-dose corticosteroids and cyclosporin with good response, although his visual acuity remains permanently impaired.