Conjunctival necrobiotic granuloma in xeroderma pigmentosum.

PURPOSE: We report clinical and histopathologic findings of a conjunctival lesion associated with xeroderma pigmentosum. METHODS: A Saudi girl with known xeroderma pigmentosum presented with pain and photophobia of the right eye and an elevated temporally located perilimbal mass. RESULTS: The mass was resected successfully and has not recurred during 1-year follow-up. Histopathologic examination of the tissue showed a necrobiotic granuloma with associated histiocytic infiltration. The patient had no systemic disease, lipid was not detected in the histiocytic lesion, no Touton giant cells were present, and there was no evidence of elastolysis. CONCLUSION: Ocular malignancies occur in association with xeroderma pigmentosum, but benign lesions that mimic a malignancy may occur.

Giant cell arteritis in Saudi Arabia.

PURPOSE: To obtain information about the incidence of giant cell arteritis in Saudi Arabia. METHODS: Retrospective review of all temporal artery biopsies performed at the King Khaled Eye Specialist Hospital from December 1982 to January 1998. RESULTS: Seventy-two temporal artery biopsies were performed over this 15 year period, of which four were positive for giant cell arteritis. CONCLUSIONS: The incidence of GCA in Saudi Arabia is probably less than in the United States and Western Europe.

Ocular histopathology in multiple endocrine neoplasia type 2b.

Multiple endocrine neoplasia type 2b is a syndrome, often familial, characterized by medullary carcinoma of the thyroid gland, pheochromocytoma, and many mucosal neuromas in patients who frequently have a marfanoid habitus. The ophthalmic findings are distinctive and include conjunctival and eyelid neuromas, keratoconjunctivitis sicca, and, most characteristically, greatly thickened corneal nerves. Ocular histopathologic study of a 30-year-old man demonstrated thickened nerves in the ciliary body, iris, and corneoscleral limbal regions. In these regions, the nerves were a mixture of myelinated and unmyelinated fibers. Sections through the thickened corneal nerves, 1.5 mm centrally from the corneoscleral limbus, showed only nonmyelinated nerves associated with Schwann cells. The many axons were normal in appearance but varied in diameter; some were between 0.1 and 0.2 ng and others between 1.0 and 1.4 ng. An unexpected finding was numerous ganglion cells in the ciliary body. The subconjunctival tissue showed neuromas with a minimum of fibrous structure. Sections of the lacrimal gland showed normal glandular tissue.

Double phakomatosis.

In the phakomatoses--von Recklinghausen's disease, the Sturge-Weber syndrome, tuberous sclerosis, and von Hippel-Lindau disease--pathologic changes in the eye are often evident. Most cases are described as a single entity. The following is a case report of concomitant Sturge-Weber syndrome and von Recklinghausen's disease in which both conditions involved the eye.