【摘要】 目的 探讨Rosai-Dorfman病的临床病理特征、诊断、鉴别诊断及治疗。
方法 对1例皮肤Rosai-Dorfman病进行组织形态学、免疫组化及特殊染色检查，并随访及复习相关文献。
结果 皮损组织真皮层见较多淋巴、浆细胞及组织细胞增生，呈片状，其间可见多核巨细胞（S-100+），并见吞噬淋巴细胞。免疫组化：CD3(++)、CD20(++)、CD79a(++)、CD68（+）、S-100（+）、CD138（+）。
结论 Rosai-Dorfman病是一种罕见的、原因不明的特发性组织细胞增生性疾病，需与朗格汉斯细胞组织细胞增生症、感染性肉芽肿、组织细胞吞噬性脂膜炎以及黄色肉芽肿等疾病相鉴别，确诊主要依靠形态学及免疫组化标记。治疗方法多种，目前意见尚未统一。
【Abstract】 Object ：To investigate the clinical and pathological featu-res, diagnosis, differential diagnosis and treatment of Rosai-Dorfman dis-ease. Methods：One cases of Rosai-Dorfman disease of skin were exa-mined by tissue morphology ,immunohistochemistry and special stainin-g ,and the related liter-atures were followed up and reviewed. Results ：Skin tissue in the der-mis see more lymph and plasma cells and tissue ce-ll hyperplasia, flaky, meantime visible multinucleated giant cells (S-100 +), and phagocytosis of lymphocytes. Immunohistochemistry: CD3 (+ +), CD20 (+ +), CD79a (+ +), CD68 (+), S-100 (+), CD138 (+). Conclusion： Rosai Dorfman disease is a rare, unexplained idiopathic organization cell hyperplasia disease, it need to differential diagnosis with Langerhans cell histiocytosis,granulo-matous infection, tissue cell phagocytosis pannicul-itis,xanthogranuloma and so on. The diagnosis mainly depends on the m-orphology and immunohistochemical markers. There are many treatme-nt methods, the current views have not been unified.
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