Juvenile xanthogranuloma of the corneal limbus: Report of two cases and review of the literature

PURPOSE: To report the clinical and histopathologic findings of limbal juvenile xanthograuloma lesions in 2 pediatric cases. METHODS: Case report and literature review. RESULTS: Two cases (a 7-month-old girl and a 7-year-old boy), both of whom developed a yellowish corneoscleral limbal mass not associated with other ocular or systemic findings, were evaluated. Both cases underwent simple excision of the lesion followed by local steroid treatment. Histopathologic examination in both cases revealed foamy histiocytes within an inflammatory infiltrate. One of the lesions also harbored multinucleate giant cells. Immunohistochemical staining was positive for CD68 and negative for S-100 and CD1a in both cases. CONCLUSIONS: Juvenile xanthogranuloma may rarely present with an isolated lesion occurring only at the corneoscleral limbus. The diagnosis in the early stages of this disorder may be established with immunophenotyping and characteristic histopathologic features even without the presence of the multinucleate giant cells.