Langerhans Cell Histiocytosis of the Uvea with a Ciliochoroidal Mass: A Case Report of Management with Systemic Therapy

Abstract

Background. This study is aimed at exploring a case of choroidal Langerhans cell histiocytosis (LCH) successfully treated with systemic corticosteroid and immunosuppressant. Case presentation. A 24-year-old man with known multisystem LCH developed loss of vision, ocular pain, conjunctival injection, panuveitis, and a ciliochoroidal mass. After receiving an intravenous methylprednisolone pulse, oral high-dose corticosteroids, and methotrexate, the mass resolved quickly and completely without flare-ups during 6 months of follow-up. Conclusions. Intraocular involvement of LCH is rare and can present with or without a history of multisystemic disease. The diagnosis is challenging, and the standard treatment is not established. Systemic anti-inflammatory and immunosuppressive therapy could be an effective treatment, as the LCH itself contains an essential element of inflammation and the symptoms may be mainly inflammatory.