Rosai-Dorfman Disease Treated with Methotrexate and Low-Dose Prednisone: Case Report and Review of the Literature

Background: Rosai-Dorfman disease is a rare condition characterized by an accumulation of histiocytes within lymph nodes and tissue. Treatment is indicated when the condition is highly symptomatic and when widespread involvement causes vital organ compression. Objective: To review the classic physical and histologic findings in Rosai-Dorfman disease and summarize the literature supporting the use of methotrexate in this condition. Methods and Results: We describe a case of Rosai-Dorfman disease that failed to respond to monotherapy with systemic corticosteroids but improved with the addition of methotrexate. Methotrexate alone or in combination with other agents has been described in at least 12 other cases in the literature and lead to a partial or complete resolution of disease in over half of these. Conclusion: The rapid improvement of clinical and physical symptoms with minimal side effects in our case and others reinforces the utility of antimetabolites in patients with Rosai-Dorfman disease who fail to respond to corticosteroids.