Iris JXG also may present as diffuse conjunctival injection with uveitis, congenital or acquired heterochromia iridis, spontaneous hyphema, or secondary glaucoma. Involvement of other uveal tissue is very uncommon. The second most commonly affected ocular site is the eyelid. Lesions appear as typical cutaneous tumors and may, on rare occasions, cause visual deprivation amblyopia. Intraocular lesions rarely have been reported in the posterior pole.
Orbital lesions are extremely uncommon.
They usually appear as infiltrative soft tissue tumors and often cause proptosis of the globe. At least one case has been described as a locally aggressive lesion causing bony destruction with intracranial extension. However, familial cases have not been observed. Development of JXG from benign cephalic histiocytosis, a related disorder, rarely has been reported. A recent report postulated this progression could be virally mediated.