Rosai-Dorfman disease manifesting as relapsing uveitis and subconjunctival masses

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy, SHML) is a rare, non-hereditary, benign histiocytic proliferative disorder mainly affecting young people with common clinical characteristics such as painless bilateral cervical lymphadenopathy, fever, leukocytosis and polyclonal gammopathy. Extranodal manifestations have been reported in 28-43% of cases. Eye involvement is relatively uncommon (8.5%), and most of cases have presented as lymphoproliferation in the soft tissues of the orbit and eyelids. Uveitis is an even more rare presentation as a review of all the literature. We describe a 63-year-old man with SHML with unusual ophthalmic manifestations of relapsing uveitis and bilateral subconjunctival masses. The results of biopsies were compatible with the characteristic histopathological findings of SHML: focal aggregations of S100-positive foamy histiocytes and the existence of lymphocytophagocytosis. During the clinical course, the patient relapsed but the relapse was relatively benign and the patient showed fair response to topical as well as systemic corticosteroid treatment. Here we describe this unusual presentation of SHML to inform physicians of the possibility for this systemic granulomatous disease to contribute to relapsing uveitis.