Abstract

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder characterized by lymphadenopathy and extra-nodal manifestations. Some patients with RDD require systemic treatment, but there is no consensus on the treatment strategy owing to its extreme rarity. Overexpression of tumor necrosis factor α (TNF-α) has been reported in lesions of patients with RDD and is thought to be involved in its pathogenesis. We herein report the first case of RDD with cutaneous involvement and arthritis that was successfully treated with methotrexate and infliximab. This case highlights the potential efficacy of anti-TNF-α therapy for RDD, offering a novel treatment option for this rare condition.

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