An unusual case of Extranodal Rosai-Dorfman Disease manifesting as a bilateral Epibulbar mass.

• Main Authors: Dr. Swati Ravani, Dr. Jaideep Sharma
• Format: Article
• Language: English
• Published: B.J.Medical College Development Society, Ahmedabad 2017-06-01
• Series: BJ Kines: National Journal of Basic & Applied Sciences
• Subjects: Epibulbar mass; Extranodal site; Rosai-Dorfman disease; Sinus histiocytosis with massive lymphadenopathy.
• Online Access: http://bjkines.com/vol9june17/paper8j17fulltext.pdf

Purpose: To report an unusual presentation of a 24-year-old man with bilateral epibulbar mass, whose presentation was confirmed histopathologically as Rosai Dorfman disease. Method: We studied a retrospective and interventional case report. Results: A 24-year-old man presented with a mass over both eyes, gradually and, progressively increasing over one year. Visual acuity and intraocular pressures were normal in both eyes. Examination revealed bilateral non tender mobile soft in consistency epibulbar mass of 3.0 x 2.0 x 0.5 cm with no overlying skin involvement. Excisional biopsy was performed. Histopathologic evaluation revealed emperipolesis, with positive CD68 (cluster of differentiation, acts as a marker for various cells of macrophage lineage), S100 (calcium binding protein believed to be glial in origin). These findings were consistent with extra nodal Rosai Dorfman disease At 6 months follow-up, there were no signs of recurrence. Conclusions: This case of Rosai Dorfman disease is rare because of the presence of bilateral epibulbar mass with no systemic involvement. It tends to be unilateral and is typically benign in nature. Excisional biopsy is often done for diagnosis and treatment. In case of unilateral Rosai Dorfman disease recurrence after biopsy is rare, but common with bilateral disease.