FOR MORE than 50 years, juvenile xanthogranuloma (JXG) has been recognized as a rare skin disease characteristically occurring in infants and young children.1 It manifests itself as benign, pink, raised lesions of the skin which often regresses spontaneously.
Ocular manifestations may occur concomitantly, or more rarely without the skin lesions.1,2 The ocular manifestations include lesions of the lids, conjunctiva, anterior uveal tract, or orbit.3 The iris lesion is the most important, having been well recognized for over ten years.2 The presenting clinical picture of uveal involvement may be one or more of the following: (1) an asymptomatic localized or diffuse iris tumor, (2) unilateral glaucoma, (3) spontaneous hyphema, (4) a red eye with signs of uveitis, or (5) congenital or acquired heterchromia iridis.
The present case is of interest because: (1) it represents the oldest known patient with this disease, (2) it presented clinically as a
Smith ME, Sanders TE, Bresnick GH. Juvenile Xanthogranuloma of the Ciliary Body in an Adult. Arch Ophthalmol. 1969;81(6):813-814. doi:10.1001/archopht.1969.00990010815011