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

IF 0.7 Q4 OPHTHALMOLOGY
Fariba Ghassemi, Hamid Riazi-Esfahani, Nazanin Ebrahimiadib, Abdulrahim Amini, Zahra Mahdizad
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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.

Abstract Image

Abstract Image

伴有纤毛脉络膜肿块的葡萄膜朗格汉斯细胞组织细胞增多症:采用全身治疗的一例报告。
背景:本研究旨在探讨一例脉络膜朗格汉斯细胞组织细胞增多症(LCH)成功治疗全身皮质类固醇和免疫抑制剂。案例演示。一名24岁男性多系统LCH患者出现视力丧失、眼痛、结膜注射、全葡萄膜炎和纤毛脉络膜肿块。在接受静脉注射甲基强的松龙脉冲、口服大剂量皮质类固醇和甲氨蝶呤后,在6个月的随访中,肿块迅速消失,没有复发。结论:LCH的眼内受累是罕见的,可伴有或不伴有多系统疾病史。诊断具有挑战性,标准治疗方法尚未建立。全身抗炎和免疫抑制治疗可能是一种有效的治疗方法,因为LCH本身含有炎症的基本成分,症状可能以炎症为主。
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