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Purpose: To report a unique case of extranodal Rosai-Dorfman disease (RDD) presenting as bilateral scleritis, which progressed to multifocal epibulbar masses.

Methods: Retrospective chart review.

Results: A 35-year-old Asian man presented with progressive pain and redness in both eyes for 4 months.

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A 29-year-old male known case of vernal keratoconjunctivitis (VKC) presented with 5-month history of a rapidly growing mass on the temporal aspect of the right cornea. The 9 × 9 mm epibulbar lesion was excised and histologically showed features of extranodal Rosai Dorfman disease (RDD) with emperipolesis of eosinophils. The lesion did not recur following excision.

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Rationale: Rosai-Dorfman disease (RDD) is a rare nonmalignant cell histiocytosis. Here, we report a rare, unusual clinical presentation of epibulbar and orbital tumor as a manifestation of RDD. We also review the literatures on clinical cases of orbital RDD.

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Background: Rosai-Dorfman disease is a rare non-Langerhans cell histiocytosis. Ocular involvement is even rarer, mostly involving the orbit and eyelids, although marginal corneal ulcers, uveitis, and epibulbar masses have also been reported, and is characterized by multiple recurrences. However, the disease course and optimal treatment strategies remain undetermined, in light of the rarity of this disease.

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Purpose: To report an unusual presentation of a 48-year-old man with a salmon-colored epibulbar mass, whose pathology was consistent with extranodal Rosai-Dorfman disease (RDD).

Method: We conducted a retrospective and interventional case report.

Results: A 48-year-old white man presented with progressive left eye irritation, redness, and multiple large subcutaneous nodules on his arms and torso over the past year.

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