Peripheral corneal degeneration and occlusive vasculitis in Wegener's granulomatosis.

A 61-year-old white woman, who had progressive arthritis over a two-year period, developed bilateral peripheral corneal degeneration. The superior cornea thinned to a thickness of approximately two thirds of normal. She died suddenly in the hospital and a primary occlusive vasculitis of the anterior ciliary arteries within the superior rectus muscles was found. There was necrotizing arteritis in the lungs, kidneys, adrenal caps, spleen, fallopian tubes, and skeletal muscle. The observation of an occlusive vasculitis of the anterior ciliary arteries in this patient supports the belief that ischemia may play a major role in peripheral marginal degeneration of the cornea.