Lymphomatoid granulomatosis presenting as sudden blindness.

Lymphomatoid granulomatosis is an uncommon clinical problem and even more rarely presents to the ophthalmologist. A case is described in which a 60-year-old Fijian woman presented with sudden, sequential, complete visual loss in association with signs of scleritis. Direct eyewall biopsy and nasal mucosal biopsy confirmed the diagnosis of lymphomatoid granulomatosis. Despite intensive high-dose oral prednisone therapy her vision remained at no light perception and she developed recrudescence of disease necessitating the use of radiotherapy. All systemic investigations failed to show any other organ involvement by the disease process and she remains well. This is the first report of lymphomatoid granulomatosis presenting as sudden blindness.