Diagnosis and management of iris juvenile xanthogranuloma.

BACKGROUND: Juvenile xanthogranuloma is a benign, self-limiting cutaneous disorder most commonly encountered during infancy. Approximately 10% of cases may develop ocular or adnexal involvement, most commonly in the iris.

METHODS: We review clinical and morphological features of four cases of iris juvenile xanthogranuloma that reflect the diagnostic 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 the iris was confirmed with a second tissue specimen obtained five months after systemic steroid treatment.

CONCLUSION: The diagnosis and treatment of juvenile xanthogranuloma may be straightforward, particularly in cases when the ocular lesion receives early attention and responds well to topical steroids, and when there is no hyphema. However, in other instances, this entity may be difficult to manage and may necessitate iris biopsy for diagnosis and radiation therapy for treatment.