Extranodal Rosai-Dorfman disease with bilateral orbital involvement: Report of a case treated with systemic steroid alone

Abstract

Safiye Yilmaz1, Melih Ture1, Ahmet Maden1, Mine Tunakan21Department of Ophthalmology, 2Department of Pathology, Izmir Atatürk Training and Research Hospital, Izmir, TurkeyAbstract: We report the case of a 41-year-old female with bilateral orbital Rosai-Dorfman disease (RDD) and the result of corticosteroid therapy. The patient developed a swollen mass of bilateral upper and lower eye-lid over a six-month period. Diagnosis was based on the characteristic histopathologic features. She was treated with oral corticosteroids 60 mg/per day for ten days and the dose of corticostreoid was tapered every week. She is currently being treated with 10 mg/per day for 4 months. In general, the prognosis is good, with experiencing remission following a course of oral corticosteroids. There is no evidence that corticosteroids are beneficial in the treatment of RDD in the literature, but data regarding such treatment are sparse. A more formalized surveillance of these cases would help to define more clearly the natural history of the disease and the effects of various treatment modalities.Keywords: Rosai-Dorfman Disease, orbital involvement, treatment, corticosteroid