Rosai-Dorfman Disease involving the eye and ocular adnexa. Clinicopathologic series of 17 patients.

Abstract

To describe the clinical features and histopathologic findings of Rosai-Dorfman disease (RDD) with ophthalmic involvement diagnosed at a Mexican ophthalmologic referral center in a period of 62 years. A total of 17 cases of RDD with ophthalmologic manifestations in Mexican-mestizo patients were collected. Female predominance was observed in 12 of 17 patients. The mean age was 36 years with a range of 12-76 years. The median age at presentation was 32 years. The orbit was the most affected location with 15 cases. Conjunctival disease was seen in 4 cases: two as isolated lesions, the rest with orbital or uveal involvement . One case suggested choroidal RDD associated to conjunctival and orbital lesion. Three cases presented multifocal disease. A patient with orbital affection developed extranodal disease elsewhere. No concurrent lymphadenopathy was observed in any patient.Histopathological examination revealed classic RDD findings in all cases: A polymorphous cellular infiltrate composed by lymphocytes, plasma cells and large histiocytes with emperipolesis hallmark of the disease. Immunohistochemically, histiocytes were positive for S-100 protein and CD68 in 15 cases. RDD of the eye and ocular adnexa in Mexican-mestizo patients is common in middle-aged women frequently as an isolated orbital lesion. Histopathology is necessary for accurate diagnosis. Most patients had a favourable outcome after medical and surgical treatment. To the best of our knowledge this is the largest case series of RDD of the eye and ocular adnexa in Latin-American patients.