皮肤Rosai-Dorfman病的皮损临床表现及组织病理特征14例分析

Clinicohistopathologic features of cutaneous Rosai-Dorfman disease:a study of 14 cases

目的:探讨皮肤Rosai-Dorfman病(CRDD)的皮损临床表现特征,结合组织病理、免疫表型和鉴别诊断可提高CRDD的正确诊断。方法:收集14例CRDD患者资料,分析其临床表现特征并进行归类分型,对标本进行组织病理和免疫组化染色观察。结果:14例CRDD患者的皮损表现可分为丘疹结节型(64.7%),浸润斑块型(5.9%)及肿瘤样型(29.4%)3种基本类型,无淋巴结增大等其他系统性病变。组织病理和免疫表型特点为真皮和皮下脂肪组织内数量不一的S-100蛋白阳性大型组织细胞,伴大量以淋巴细胞和浆细胞为主的混合性炎性细胞浸润,组织细胞胞质内常吞有数量多少不一的淋巴细胞。1例脉管内可见组织细胞。少数患者出现淋巴滤泡、纤维化及脂膜炎样和血管炎样组织病理形态。结论:CRDD皮损表现多种多样,3种基本皮损类型的分型方法能提高临床对CRDD诊断的皮损鉴别能力,结合组织病理改变和免疫表型可与多种皮肤病和皮肤肿瘤作鉴别。CRDD可复发,应作长期随访。

Objective： To improve the diagnosis of cutaneous Rosai-Dorfman disease （CRDD） by investigating the clinical, pathological and immunophenotypic features of this disease. Methods： The data of 14 cases of CRDD were collected. The clinical category was classified according the features of clinical manifestations of these patients. The histopathologic and immunohistochemical studies were performed in all cases. Results： The skin lesions of 14 patients were divided into 3 main types： papulonodular type （64.7%）, infiltrating plaque type （5.9%） and tumor type （29.4%）. No other systemic disease such as lymphadenopathy was found. Mixed inflammatory cell infiltration in the dermis and subcutis, mainly lymphocytes, plasma cells, and a variable number of S-100 positive histiocytes, was shown by histopathology and immunohistochemistry. The presence of lymphocytes engulfed within the histiocytic cytoplasm, namely cmperipolesis, was seen. Few histiocytes between vascular spaces were found in one case. Lymph follicle, fibrosis, panniculitis-like change and vaseulitis-like change were seen in a few cases. Conclusions： The classification of the skin lesions into three types, combined with histopathologic and immunophenotypic features, are helpful for the diagnosis of CRDD, especially for the differentiation of it from various other cutaneous neoplastic and non-neoplastic skin diseases.