ABSTRACT

Purpose

To report a unique case of extranodal Rosai-Dorfman disease (RDD) presenting as bilateral scleritis, which progressed to multifocal epibulbar masses.

Methods

Retrospective chart review.

Results

A 35-year-old Asian man presented with progressive pain and redness in both eyes for 4 months. Examination revealed bilateral diffuse scleral congestion and areas of scleral thinning. The cornea and anterior chamber of each eye were unremarkable. Despite oral corticosteroid and immunosuppressive treatment, scleritis progressed to multifocal epibulbar masses. Tissue biopsy specimens revealed foamy macrophages with histiocytes staining positive for CD68 and S100, confirming a diagnosis of RDD. Systemic evaluation was unremarkable for lymphadenopathy or extranodal involvement. The patient was started on systemic chemotherapy and at last follow up after 1 year, all epibulbar lesions were completely resolved.

Conclusions

Rosai-Dorfman disease can present as bilateral scleritis initially and develop epibulbar masses without any systemic involvement. Biopsy with immunohistochemical analysis can aid in the diagnosis.

中文翻译：

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双侧眼部 Rosai-Dorfman 病表现为弥漫性前巩膜炎伴多发性扩大的眼睑肿块

摘要

目的

报告一例独特的结外 Rosai-Dorfman 病 (RDD) 病例，表现为双侧巩膜炎，并进展为多灶性球外肿块。

方法

回顾性图表审查。

结果

一名 35 岁的亚洲男性因双眼进行性疼痛和发红 4 个月就诊。检查发现双侧弥漫性巩膜充血和巩膜变薄区域。每只眼睛的角膜和前房均无异常。尽管口服皮质类固醇和免疫抑制治疗，巩膜炎仍进展为多灶性球外肿块。组织活检标本显示泡沫状巨噬细胞和组织细胞 CD68 和 S100 染色阳性，证实了 RDD 的诊断。系统评估未发现淋巴结肿大或结外受累。患者开始全身化疗，1年后随访，球上病灶全部完全消退。

结论

Rosai-Dorfman 病最初可表现为双侧巩膜炎，并在没有任何全身受累的情况下发展为球外肿块。免疫组织化学分析活检有助于诊断。