Extranodal marginal zone lymphoma associated with Vogt-Koyanagi-Harada disease

This case report highlights a potential association between ocular lymphoma and recurrent uveal inflammation secondary to Vogt-Koyanagi-Harada (VKH) disease. A 61-year-old male with history of bilateral panuveitis was referred for management of exudative retinal detachment in the left eye (OS). Visual acuity was 20/20 right eye (OD) and light perception OS. Funduscopic examination was significant for pigmentary changes OD and inferior exudative retinal detachment OS. The patient was diagnosed with VKH disease and was treated with topical steroids and mycophenolate mofetil. He later presented acutely with reduced vision to no light perception OS and intraocular pressure of 71 mmHg OS. A B-scan ultrasound OS showed presence of a posterior mass with an overlying retinal detachment and choroid thickening. Enucleation was performed. Histologic findings confirmed extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (EMZL). We hypothesize that EMZL may have evolved in the context of chronic and recurrent uveal inflammation.