BACKGROUND: Juvenile xanthogranuloma is an uncommon benign skin disease primarily affecting infants and young children. Ocular involvement is rare and most often affecting the iris. Isolated xanthogranulomas of the corneoscleral limbus are reported in the literature in only a few patients. HISTORY AND FINDINGS: A 10-year-old boy presented in October 1993 with a yellow solid tumor at the limbus of his left eye. The other ocular and extraocular structures were normal. Furthermore, he showed a preauricular nodular skin lesion. THERAPY AND OUTCOME: We suspected a dermoid of the corneoscleral limbus and performed an excision of the tumor. Histological examination revealed a juvenile xanthogranuloma. The tumor had regrown to its original size 2 months after excision. Treatment with topical corticosteroids resulted in an acceptable but incomplete regression of the tumor. CONCLUSION: Juvenile Xanthogranulomas of the corneoscleral limbus are rare tumors. The majority of previously reported cases showed a recurrence of the tumor at the original site after simple excision. In the case of unsuccessful therapy with topical corticosteroids a lamellar graft is recommended.
BACKGROUND:Juvenile xanthogranuloma is an uncommon benign skin disease primarily affecting infants and young children. Ocular involvement is rare and most often affecting the iris. Isolated xanthogranulomas of the corneoscleral limbus are reported in the literature in only a few patients. HISTORY AND FINDINGS: A 10-year-old boy presented in October 1993 with a yellow solid tumor at the limbus of his left eye. The other ocular and extraocular structures were normal. Furthermore, he showed a preauricular nodular skin lesion. THERAPY AND OUTCOME: We suspected a dermoid of the corneoscleral limbus and performed an excision of the tumor. Histological examination revealed a juvenile xanthogranuloma. The tumor had regrown to its original size 2 months after excision. Treatment with topical corticosteroids resulted in an acceptable but incomplete regression of the tumor. CONCLUSION: Juvenile Xanthogranulomas of the corneoscleral limbus are rare tumors. The majority of previously reported cases showed a recurrence of the tumor at the original site after simple excision. In the case of unsuccessful therapy with topical corticosteroids a lamellar graft is recommended.