皮肤Rosai-Dorfman病的研究进展

Cutaneous Rasai-Dorfman disease

Rosai-Dorfman病是一种罕见的组织细胞增生性疾病,病因及发病机制尚不明确,可能与感染和免疫功能紊乱引起的信号转导通路激活及炎症反应有关。仅有皮肤受累者称为皮肤Rosai-Dorfman病。皮损组织病理可见真皮大量组织细胞伴淋巴细胞、浆细胞浸润,在组织细胞的胞浆内可见吞噬的完整淋巴细胞,称为伸入运动。免疫组化示组织细胞阳性表达CD68和S100,不表达CD1a。CRDD的治疗可采用手术切除、局部治疗及系统治疗,部分患者皮疹可自行消退,预后较好。

Rosai-Dorfman disease(RDD)is a rare histiocytic proliferative disorder.The etiology and pathogenesis of RDD are still unclear,and may be associated with the activation of signal transduction pathways and inflammatory reactions induced by infection and immune dysfunction.CRDD is defined as the RDD involving only the skin.Histopathological examination of CRDD lesions shows that a large amount of histiocytes,lymphocytes and plasma cells infiltrate the dermis,and intact endocytosed lymphocytes can be observed in the cytoplasm of histiocytes,which is called emperipolesis.Immunohistochemical study shows that histiocytes are stained positive for S-100 and CD68,but negative for CD1a.The treatment of CRDD includes surgical excision,local therapy and systemic therapy.Lesions can subside spontaneously in some patients,and it usually has a favorable prognosis.