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Mark W. Johnson, MD; Gregory L. Skuta, MD; Marilyn C. Kincaid, MD; Christine C. Nelson, MD; J. Reimer Wolter, MD

Arch Ophthalmol. 1989;107(3):402-407.

Abstract
• Xeroderma pigmentosum is an autosomal recessive, precancerous dermatosis caused by defective repair of ultraviolet-damaged DNA. Characterized clinically by progressive cutaneous pigmentary alterations and tumorigenesis, it serves as a model for ultraviolet carcinogenesis. We describe the clinical and histopathologic findings in a 31-year-old woman with xeroderma pigmentosum and a massive iris melanoma of the left eye. Histologic examination following enucleation revealed diffuse iris replacement by spindle and epithelioid cells with extension into the trabecular meshwork. Evidence of direct extraocular extension was absent, and a metastatic evaluation showed no abnormalities. To our knowledge, this is the first reported case of xeroderma pigmentosum complicated by melanoma of the iris. It provides further evidence suggesting a role for sunlight exposure in the pathogenesis of uveal melanoma.

Author Affiliations
From the W. K. Kellogg Eye Center, the Departments of Ophthalmology (Drs Johnson, Skuta, Nelson, and Wolter) and Pathology (Dr Wolter), University of Michigan, Ann Arbor; and the Department of Pathology, Henry Ford Hospital, Detroit (Dr Kincaid).

Footnotes
Accepted for publication Sept 16, 1988.

Read in part before the Annual Meeting of the Association for Research in Vision and Ophthalmology, Sarasota, Fla, May 5, 1988.

Reprint requests to W. K. Kellogg Eye Center, 1000 Wall St, Ann Arbor, MI 48105 (Dr Skuta).

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