Juvenile xanthogranuloma masquerading as melanoma. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus [J AAPOS] Journal article

Juvenile xanthogranuloma (JXG) of the uvea is a rare disease that usually responds to systemic steroids or low-dose radiotherapy. We present an atypical case of bilateral JXG involving the entire uveal tract that presented with an aggressive phenotype. The patient was unresponsive to topical and systemic corticosteroids, cyclosporine, and maximal doses of radiation therapy. The disease was ultimately controlled with the alkylating agent chlorambucil.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus / American Association for Pediatric Ophthalmology and Strabismus [J AAPOS]