Abstract
BackgroundRosai-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.Case presentationWe reported a 36-year-old male patient with the extranodal form of Rosai-Dorfman disease, presenting with scleritis and anterior uveitis in the left eye, who experienced subsequent development of an epibulbar tumor in the same eye. The patient was also complicated by a relapsing facial nodule on the right cheek. After the pathological diagnosis of Rosai-Dorfman disease was obtained, the patient underwent surgical excision of the epibulbar tumor and the facial nodule, accompanied by systemic immunosuppression therapy. At the last follow-up, the patient was asymptomatic without signs of recurrence.ConclusionsThis report highlights the progression of ocular manifestations of Rosai-Dorfman disease and emphasizes the importance of systemic therapy.
Highlights
Rosai-Dorfman disease is a rare non–Langerhans cell histiocytosis
This report highlights the progression of ocular manifestations of Rosai-Dorfman disease and emphasizes the importance of systemic therapy
Rosai-Dorfman disease (RDD) is a rare non–Langerhans cell histiocytosis that is characterized by the accumulation of activated histiocytes within affected tissues, and it usually presents with prominent cervical lymphadenopathy [1]
Summary
Rosai-Dorfman disease is a rare non–Langerhans cell histiocytosis. Ocular involvement is even rarer, mostly involving the orbit and eyelids, marginal corneal ulcers, uveitis, and epibulbar masses have been reported, and is characterized by multiple recurrences. Conclusions: This report highlights the progression of ocular manifestations of Rosai-Dorfman disease and emphasizes the importance of systemic therapy. We report an unusual case of RDD with ocular and dermatological findings, including scleritis and anterior uveitis and the subsequent development of an epibulbar tumor, and was complicated by a relapsing facial nodule. At the 24-month follow-up, there were no signs of recurrence of the epibulbar tumor and facial mass or of involvement of other sites (Fig. 1d).
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