皮肤窦组织细胞增生症伴巨大淋巴结病的研究进展

Cutaneous Rosai-Dorfman Disease

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、皮肤斑块、结节,伴有白细胞增多、高γ球蛋白血症、血沉增快。仅有皮肤损害,无其他器官受累者称为皮肤Rosai-Dorfman病。皮损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。部分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 Histiocytosis with massive lymphadenopathy （SHML） or rosai-dorfman disease is a benign, histiocytic proliferative disorder with undetermined etiology. It usually appears as a febrile illness associated with massive cervical lymphadenopathy, skin lesions, hyperglobulinemia, leukocytosis and an elevated sedimentation rate. 10% of the patients with SHML have skin lesions, patients with only skin lesions were called ＂cutaneous rosai-dorfman disease＂ which manifestated as yellow-brown papules, nodules, and plaques. A very diagnostic feature in histopathology is the finding of intact lymphocyte （or plasma cells） in the cytoplasm of the histiocytic cells which was called ＂emperipolesis＂. The lesions may clear spontaneously in some patients, treatment of RDD include surgical excition, systemic corticosteroids, thalidomied and retinoids.