[Peripheral ulcerative keratitis and a form of limited wegener's granulomatosis]

A man aged 68 years presents superior limbal infiltrates at his left eye two weeks before a marginal ulcer which quickly perforates. He has no systemic complaint. Clinical, biological, radiologic and histological evaluations disclose superior airways and lungs implications, an inflammatory syndrome, high ANCA (antineutrophiles cytoplamic antibodies) titer and vasculitis. There is no sign of renal involvement. A limited form of Wegener's granulomatosis is diagnosed. The outcome is favorable with a partial penetrating keratoplasty and systemic corticosteroid therapy in association with immunosuppressive drugs. This so called limited form of Wegener's granulomatosis is sight threatening when eye is the initial presentation. The early diagnostic and treatment will be performed by the help of ANCA in cases with subclinical systemic manifestations.