Abstract： Rosai-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.