窦组织细胞增生症伴巨大淋巴结病是一种病因不明的良性组织细胞增生性疾病,又称Rosai-Dorfman病。可累及多个器官,临床表现为发热、颈部淋巴结肿大、皮肤黄瘤样斑、结节,伴有白细胞增多、高球蛋白血症、血沉增快。组织病理可见真皮大量组织细胞浸润,在组织细胞的胞浆内可见被吞噬的完整的淋巴细胞、嗜中性粒细胞等。患者皮疹可自行消退,治疗可采用手术切除、口服糖皮质激素、反应停、维甲酸等。 Sinus cell histiocytosis with giant lymph node disease is an unknown etiology of benign tissue cell proliferative disease, also known as Rosai-Dorfman disease. May involve multiple organs, clinical manifestations of fever, neck lymph nodes, skin xanthomatosis, nodules, associated with leukocytosis, hypergammaglobulinemia, ESR faster. Histopathology showed a large number of dermis tissue cells infiltration in the cytoplasm of tissue cells can be swallowed intact lymphocytes, neutrophils and so on. Patients with rash can subside on their own, the treatment can be surgical resection, oral glucocorticoid, reaction stop, retinoic acid and so on.