Langerhans cell histiocytosis with hemorrhagic uveitis and exudative retinal detachment

Abstract

Bilateral intraocular involvement in Langerhans cell histiocytosis (LCH) is uncommon. A 15-year-old boy presented with painless decreased vision in right and painful left red eye of 2 weeks duration. Visual acuity was 20/500 and 20/200, respectively. A fixed dilated pupil with exudative retinal detachment was present in the right eye and hemorrhagic iris nodules with hyphema and hypopyon were seen in the left eye. Intraocular pressure was 12 and 31 mmHg, respectively, in each eye. Ocular symptoms were preceded by fever with multiple skin rashes, subcutaneous nodules, and lymph node enlargement. The histopathological examination of skin and lymph node showed histiocytes positive for histiocytic S 100. He was treated with topical steroids and antiglaucoma eye drops along with intravenous vinblastine 6 mg and oral prednisolone (1 mg/kg). Hyphema and hypopyon were resolved, vision improved to 20/320 and 20/80, and intraocular pressure was under control. However, as multisystemic LCH has a poor prognosis, we were unable to save him. Hence, fatal conditions like LCH should also be considered in the differential diagnosis of any hemorrhagic uveitis.