Epibulbar rosai-dorfman disease: novel manifestation and treatment.

S inus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease [RDD]) is a benign, idiopathic, self-limiting lymphoproliferative disease, described by Rosai and Dorfman in 1969. Extranodal manifestations represent about 43% of cases. The disease is classically accompanied by fever, malaise, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. Rosai-Dorfman disease is diagnosed histopathologically, with abundant histiocytes often engulfing lymphocytes and lymphoid cells, known as emperipolesis. The histiocytes are characteristically reactive to CD68 and S-100 proteins. Herein, we discuss the unique case and treatment of a woman who had RDD with bilateral epibulbar lesions concurrent with anterior uveitis.