Juvenile xanthogranuloma: concurrent involvement of skin and eye

To report a novel presentation of ocular involvement in juvenile xanthogranuloma (JXG).

A 1-year-old boy with the concurrent skin and eye involvement of JXG is presented. The ocular problems included spontaneous hyphema, secondary glaucoma, and 360-degree involvement of the limbus, iris, and angle.

To reduce the intraocular pressure (IOP), treatment was started with drops of timolol, betamethasone, and atropine and acetazolamide suspension. After 6 months of medical treatment, the skin lesions did not change, but the iris lesion enlarged without hyphema. The IOP in the right eye was controlled by the medications.

Although JXG is an uncommon disease, it is one of the most important causes of spontaneous hyphema in children. Iris involvement, spontaneous hyphema, and secondary glaucoma are the most common ocular problems. Corneal involvement often is characterized by a yellowish protrusion of the limbus along with blood staining of recurrent hyphema and elevated IOP. In this report, we describe an unusual case of JXG with 360-degree limbus, iris, and angle involvement. To our knowledge, this is the first report in the medical literature of 360-degree limbus involvement in JXG.