摘要Rosai-Dorfman病是一种少见的累及淋巴结的皮肤良性炎症性组织细胞增生症,组织病理以大量胞质丰富的组织细胞增生伴伸入运动,免疫组化染色CD68、S100阳性为特征.该病的起源及机制尚不清楚,目前认为,最可能的机制是巨噬细胞集落刺激因子诱导分化免疫抑制性巨噬细胞、淋巴细胞活化受阻以及大量细胞因子,包括核因子κB、肿瘤坏死因子d、白细胞介素1β、白细胞介素6及多种细胞趋化因子受体分泌增加.同时人疱疹病毒感染、恶性肿瘤、SLC29A3基因突变可能参与发病.Rosai-Dorfman病的病程通常自限性,当病变广泛或出现系统症状,一般以手术切除、口服糖皮质激素、放疗或化疗进行治疗.

abstractsRosai-Dorfman disease is a rare benign inflammatory cutaneous histiocytosis with the involvement of lymph nodes.It is histopathologically characterized by proliferation and emperipolesis of histiocytes with abundant cytoplasm that stain positive for CD68 and S100.The origin and pathogenesis of Rosai-Dorfman disease are unclear at present,and the most probable pathogeneses are that macrophage colonystimulating factor induces the differentiation of immunosuppressive macrophages,hampers the activation of lymphocytes,and increases the expressions of multiple cytokines,including nuclear factor kappa B,tumor necrosis factor-alpha,interleukin-1β (IL-1β),IL-6 and various chemokine receptors.In addition,human herpes virus infection,malignant tumors and mutations in the SLC29A3 gene may contribute to the occurrence of Rosai-Dorfman disease.It usually undergoes spontaneous regression.Surgical excision,oral glucocorticoids,radiotherapy and chemotherapy are often used when lesions are extensive or systemic symptoms appear.