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

Abstract

Purpose 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. Results 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. Conclusion 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.

Rosai–Dorfman disease as chronic bilateral granulomatous anterior uveitis: A case report

“…There were variously reported tailored approaches, including observation in asymptomatic cases or management with the different routes of corticosteroids (topical, intravitreal, and systemic administration), surgery, radiation, and chemotherapy in mass lesions or systemic involvement. 4 , 6 – 8 , 12 – 15 , 17 – 21 RDD associated with uveitis tends to be completely cured after treatment ( Table 1 ). Even though, in our case, the patient had moderate NPDR and CME might be a consequence of this disease, we believed this CME was uveitic macular edema rather than diabetic macular edema.…”

“… 3 – 5 An orbital mass and epibulbar mass were commonly reported presentations. 4 – 7 An intraocular involvement of RDD is exquisitely rare, but it tends to have more aggressive characters and multiple organ involvement. 4 Most reported cases presented with mass lesions, such as ciliary body mass and choroidal mass.…”