Dpartment of Pathology and Division of Dermatology, Centre Hospitalier de l'Université de Montréal, QC.
J Cutan Med Surg. 2012 Jul-Aug;16(4):281-5. doi: 10.1177/120347541201600411.
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.
To review the classic physical and histologic findings in Rosai-Dorfman disease and summarize the literature supporting the use of methotrexate in this condition.
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.
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.
罗道氏病（Rosai-Dorfman disease）是一种罕见疾病，其特征是组织和淋巴结中组织细胞的积累。当病情高度明显且广泛涉及导致重要器官受压时，需要进行治疗。
回顾罗道氏病的典型体格检查和组织学发现，并总结支持在这种情况下使用甲氨蝶呤的文献。
我们描述了一例罗道氏病病例，该病例对全身性皮质类固醇单药治疗无反应，但加用甲氨蝶呤后有所改善。文献中至少有 12 例其他病例描述了单独使用甲氨蝶呤或与其他药物联合使用，其中超过一半的病例的疾病部分或完全缓解。
我们的病例和其他病例中，临床和身体症状迅速改善，副作用最小，这再次证实了在对皮质类固醇无反应的罗道氏病患者中使用抗代谢物的效用。
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