Necrotizing perilimbal ulceration of the sclera and cornea has been studied clinically and histopathologically in both eyes of a patient in whom the clinical diagnosis of Wegener's granulomatosis was confirmed at autopsy. While corneoscleral ulceration is not entirely specific, and has been described also in patients with the closely related complex of periarteritis nodosa, it is, nevertheless, a characteristic clinical sign of the general category of diseases to which Wegener's granulomatosis belongs and its recognition, by the ophthalmologist, may be of considerable importance. The present study indicates that the ocular changes occurring in diseases of this group are not histopathologically identical. Although no previous study of the ocular pathology in Wegener's granulomatosis is available for comparison, necrotizing granulomata of the ciliary body seem to be a distinctive feature of this disease and one which has not been reported in periarteritis nodosa.
A number of excellent reviews of the systemic clinical
FRAYER WC. The Histopathology of Perilimbal Ulceration in Wegener's Granulomatosis. AMA Arch Ophthalmol. 1960;64(1):58-64. doi:10.1001/archopht.1960.01840010060004