Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Japan.
Division of Rheumatology, Department of Medicine, National Hospital Organization Tokyo Medical Center, Japan.
Intern Med. 2024 Nov 1;63(21):2991-2994. doi: 10.2169/internalmedicine.3295-23. Epub 2024 Mar 4.
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.
罗萨达-多夫曼病（RDD）是一种罕见的组织细胞增生性疾病，其特征为淋巴结病和结外表现。一些 RDD 患者需要全身治疗，但由于其极为罕见，因此对于治疗策略尚无共识。据报道，RDD 病变中肿瘤坏死因子 α（TNF-α）表达过度，被认为参与其发病机制。本文报告首例伴有皮肤受累和关节炎的 RDD 病例，经甲氨蝶呤和英夫利昔单抗治疗后成功缓解。该病例提示抗 TNF-α 治疗 RDD 的潜在疗效，为这种罕见疾病提供了一种新的治疗选择。
