Lee Yu-Kuei, Chao Sheau-Chiou, Lee Chaw-Ning, Hung Jia-Horung
Department of Ophthalmology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.
BMC Ophthalmol. 2019 Jul 10;19(1):144. doi: 10.1186/s12886-019-1158-2.
Rosai-Dorfman disease is a rare non-Langerhans cell histiocytosis. Ocular involvement is even rarer, mostly involving the orbit and eyelids, although marginal corneal ulcers, uveitis, and epibulbar masses have also been reported, and is characterized by multiple recurrences. However, the disease course and optimal treatment strategies remain undetermined, in light of the rarity of this disease.
We reported a 36-year-old male patient with the extranodal form of Rosai-Dorfman disease, presenting with scleritis and anterior uveitis in the left eye, who experienced subsequent development of an epibulbar tumor in the same eye. The patient was also complicated by a relapsing facial nodule on the right cheek. After the pathological diagnosis of Rosai-Dorfman disease was obtained, the patient underwent surgical excision of the epibulbar tumor and the facial nodule, accompanied by systemic immunosuppression therapy. At the last follow-up, the patient was asymptomatic without signs of recurrence.
This report highlights the progression of ocular manifestations of Rosai-Dorfman disease and emphasizes the importance of systemic therapy.
罗萨伊-多夫曼病是一种罕见的非朗格汉斯细胞组织细胞增生症。眼部受累更为罕见，主要累及眼眶和眼睑，尽管也有边缘性角膜溃疡、葡萄膜炎和眼球表面肿物的报道，且其特点是多次复发。然而，鉴于这种疾病的罕见性，其病程和最佳治疗策略仍未确定。
我们报告了一名36岁的男性患者，患有结外型罗萨伊-多夫曼病，左眼出现巩膜炎和前葡萄膜炎，随后同一只眼睛出现了眼球表面肿瘤。该患者还伴有右侧脸颊复发性面部结节。在获得罗萨伊-多夫曼病的病理诊断后，患者接受了眼球表面肿瘤和面部结节的手术切除，并辅以全身免疫抑制治疗。在最后一次随访时，患者无症状，无复发迹象。
本报告强调了罗萨伊-多夫曼病眼部表现的进展，并强调了全身治疗的重要性。
