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Case Report
9 (
1
); 50-55

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

Associate Professor & Head of occuloplasty unit, M & J western regional institute of ophthalmology, Ahmedabad.
Resident doctor, M & J western regional institute of ophthalmology, Ahmedabad.

*Corresponding Author: Dr Jaideep Sharma E-mail: jaideep7007@gmail.com

Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

Abstract

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.

Keywords

Epibulbar mass
Extranodal site
Rosai-Dorfman disease
Sinus histiocytosis with massive lymphadenopathy

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