DIAGNOSIS AND MANAGEMENT OF IRIS JUVENILE XANTHOGRANULOMA

Background: Juvenile xanthogranuloma is a benign, self-limiting cutane ous disorder most commonly encountered during infancy. Approximately 1 0% of cases may develop ocular or adnexal involvement, most commonly i n the iris. Methods: We review clinical and morphological features of four cases of iris juvenile xanthogranuloma that reflect the diagnosti c and therapeutic spectrum. Results: Tissue diagnosis was confirmed in all cases; in one case, the disease was diagnosed with a skin biopsy and treated with local and systemic steroids, and its persistence in t he iris was confirmed with a second tissue specimen obtained five mont hs after systemic steroid treatment. Conclusion: The diagnosis and tre atment of juvenile xanthogranuloma may be straightforward, particularl y in cases when the ocular lesion receives early attention and respond s well to topical steroids, and when there is no hyphema. However, in other instances, this entity may be difficult to manage and may necess itate iris biopsy for diagnosis and radiation therapy for treatment.