皮肤Rosai-Dorfman病2例报告并文献复习

Cutaneous Rosai-Dorfman disease:two cases reports and review of the literature

目的探讨皮肤Rosai-Dorfman病(CRDD)的临床病理学特征、免疫学表型和鉴别诊断。方法分析2例CRDD患者的临床表现,对标本进行病理学和免疫组织化学染色观察并文献复习。结果 CRDD镜下病理特征为真皮层较多的体积较大的组织细胞浸润,呈多边形或椭圆形,胞质丰富,淡嗜酸性,胞浆内吞噬有数量不等的淋巴细胞、浆细胞或其他炎细胞,免疫组化染色显示组织细胞表达S-100蛋白、CD68,但CD1a和CD34表达阴性。结论 CRDD病是一种少见的组织细胞增生性病变,在病理诊断方面,易误诊为其他疾病,应与其他组织细胞增生性疾病相鉴别。

Objective To investigate the clinicopathologic feature, immunophenotype and differential diagnosis of cu- taneous Rosai-Dorfman disease （CRDD）. Methods Clinical manifestation, morphologic features and immunohisto- chemical staining were studied in 2 cases of CRDD, along with a literature review. Results The lesions were charac- terized by the presence of large histiocytes with emperipolesis, accompanied by infiltration of lymphocytes, plasma cells and other inflammatory cells. The large histiocytes had an abundant cytoplasm, pale to eosinophilic in appear- ance. Immunohistochemcal examination showed expression of S-100 protein and CD68 on the histiocytes, but no CDla and CD34. Conclusions CRDD is known as an idiopathic proliferative disease of histiocytes and is very rare. And it is liable to be misdiagnosed in pathologic diagnosis. Differential diagnosis from other types of fibrohistiocytic proliferation lesions is recommended.