Intraocular juvenile xanthogranuloma of the iris in an adult patient

Abstract

PurposeJuvenile xanthogranuloma (JXG) is a rare histiocytic skin disease primarily of young children, which may also affect ocular structures and in particular the iris.ObservationsThis is a case report of a fifty-year-old patient without skin lesions showing a progressive decrease of visual acuity, iris vascularization and a yellowish iris tumor in the iridocorneal angle of his right eye. Treatment with topical and systemic prednisolone led to full recovery of visual acuity, tumor regression and restitutio ad integrum.Conclusions and importanceAlthough the juvenile xanthogranuloma is a very rare skin disease of young children, it may also affect the eye and in particular the iris in adult patients. There exists no standard treatment, the first-line therapy in most cases, however, is topical and systemic prednisolone application.

Adult onset xanthogranuloma of the eyelid

“…Two case series concerning eye involving JXG include patients with eyelid involvement presenting in their third and fifth decades, however these do not elaborate on the clinical features of these presentations. 3 , 22 Several individual case reports have discussed adult-onset JXG arising in other ophthalmic structures including the iris, 23 limbus 24 , 25 , 26 , 27 and orbit. 28 , 29 , 30 …”

“…It is characterized histologically by an infiltrate of epithelioid to foamy histiocytes and multinucleate Touton giant cells. 1 Typically, lesions affect the skin, however the eye is the most frequently affected extra-cutaneous site. 2 Ophthalmic involvement can occur at the orbit, eyelid, iris, retina, choroid and the optic nerve, with the majority of cases being diagnosed within the first year of life.…”

Complete resolution of iris juvenile xanthogranuloma in an infant with topical corticosteroid treatment only

“…Treatments for iris JXG have included corticosteroids (topical, subtenon, and systemic administration), radiation therapy, methotrexate, intravitreal bevacizumab, and surgical excision. 4,5 Treatment and complete resolution of iris JXG exclusively with topical corticosteroids has not been described previously. Our patient's classic presentation in infancy with an ochre-yellow vascular iris lesion, brisk anterior chamber reaction, and negative hematologic and ultrasound features allowed us to overlook the important guideline that tissue diagnosis is mandatory for any iris lesion prior to treatment.…”