Bilateral Ocular Rosai-Dorfman Disease Presenting as Diffuse Anterior Scleritis with Multiple Enlarging Epibulbar Masses.

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.