罗赛-多夫曼病 (RDD) 是一种罕见的非朗格汉斯细胞组织细胞增多症，其特征是淋巴结内组织细胞增殖。可发生结外受累；然而，只有 10% 的结外 RDD 涉及皮肤。我们介绍了一名 66 岁女性的独特病例，她患有皮肤 RDD，随后发展为多发性骨髓瘤 (MM)。据我们所知，这只是第二例报告的 RDD 先于 MM 诊断的病例，第一个有记录的病例发生在 2018 年。该患者到皮肤科诊所就诊，右脸颊有 5 年无痛、孤立性病变。 。局部检查发现右侧鼻唇沟和脸颊上有一个 6 毫米 x 7 毫米、边界清楚的珍珠状毛细血管扩张性病变，类似于基底细胞癌。切除病灶，保留 3 毫米的圆周边缘。组织病理学显示混合淋巴组织细胞浸润并具有伸入现象，免疫组织化学染色模式与 RDD 一致。两年后，患者出现髋部疼痛，并被诊断为 MM。她接受了来那度胺、硼替佐米和地塞米松治疗，随后继续服用来那度胺。我们的案例增加了表明 RDD 和 MM 之间潜在关联的有限证据。未来需要在这一领域进行进一步研究，以便及时识别和管理出现此类表现的患者。© 2024 作者：

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis disorder characterized by the proliferation of histiocytes within the lymph nodes. Extranodal involvement can occur; however, only 10% of extranodal RDD involve the skin. We present a unique case of a 66-year-old woman with cutaneous RDD followed by the development of multiple myeloma (MM). To our knowledge, this is only the second reported case where RDD preceded a diagnosis of MM, with the first documented instance occurring in 2018. The patient presented to the dermatology clinic with a 5-year history of painless, solitary lesion over the right cheek. Local examination revealed a single 6 mm x 7 mm well-circumscribed pearly telangiectatic lesion resembling basal cell carcinoma over the right nasolabial fold and cheek. The lesion was excised with a 3 mm circumferential margin. Histopathology showed a mixed lymphohistiocytic cell infiltrate with emperipolesis and immunohistochemical staining patterns consistent with RDD. Two years later, the patient presented with hip pain and was diagnosed with MM. She was treated with lenalidomide, bortezomib, and dexamethasone, and was later maintained on lenalidomide. Our case adds to the limited evidence suggesting a potential association between RDD and MM. Further research in this field is required to promptly identify and manage patients with such a presentation in the future.© 2024 by the authors.