An unusual case of juvenile xanthogranuloma presenting as a subconjunctival nodule with review of literature

Abstract

Juvenile xanthogranuloma (JXG) is a benign histiocytic disorder that belongs to the non-Langerhans cell group. Extracutaneous involvement in the eye usually involves the iris and presents as recurrent spontaneous episodes of hyphema or secondary glaucoma. A 3-year-old child presented with a subconjunctival mass in the left eye and concurrent scalp nodules. Ultrabiomicroscopic examination showed a moderately reflective episcleral mass with extension into cornea but no scleral involvement. Histopathology of the excised subconjunctival mass showed a dense infiltrate of polygonal/spindle mononuclear cells with moderate amounts of cytoplasm and scattered Touton giant cells, suggestive of JXG. Systemic evaluation did not reveal any other sites of involvement. Postoperatively, the patient was treated with topical steroids. At last follow-up 2.5 years, the patient is free on any recurrence.