皮肤Rosai-Dorfman病40例临床及病理分析  

Clinical and Histopathological Analysis of 40 Cases with Cutaneous Rosai-Dorfman Disease

作　　者：肖翠容 陈凤鸣[1] 王雷[1] XIAO Cuirong;CHEN Fengming;WANG Lei(Department of Dermatology,Xijing Hospital,Fourth Military Medical University,Xi'an 710032,China;Department of Dermatology,Zhangzhou Affiliated Hospital of Fujian Medical University,Zhangzhou 363000,China)

机构地区：[1]第四军医大学西京皮肤医院,陕西西安710032 [2]福建医科大学附属漳州市医院皮肤科,福建漳州363000

出　　处：《中国皮肤性病学杂志》2021年第8期876-881,共6页The Chinese Journal of Dermatovenereology

摘　　要：目的探讨皮肤Rosai-Dorfman病(cutaneous Rosai-Dorfman disease,CRDD)的临床表现结合组织病理特征及免疫组化,以期提高临床医师对CRDD的诊断率。方法收集40例CRDD患者的临床及病理资料,分析不同分型皮损的临床及组织病理学表现,总结不同皮损的组织病理学特征、治疗和预后。结果40例患者中女25例(62.50%),男15例(37.50%);26例为单发(65.00%),14例为多发(35.00%);32例行单处组织病理检查,8例行2处组织病理检查,共48处皮损,其中丘疹结节型25处(52.08%)、浸润斑块型18处(37.50%)和肿瘤样型5处(10.42%)。40例患者中,随访34例(85.00%),失访6例(15.00%),随访期为6~66个月。22例接受不同治疗。12例未接受治疗者中,2例出现新发皮损,但原部位皮损较前变平、变小。组织病理示:真皮全层及皮下组织交替出现淡染区及深染区,可见数量不一、体积较大的组织细胞,组织细胞内见淋巴细胞、浆细胞及中性粒细胞被吞噬的现象,可伴有血管数量增加、胶原纤维化及淋巴滤泡等不同表现。免疫组织化学显示所有病例中组织细胞均表达S100和CD68,不表达CD1a。结论CRDD的临床表现是以丘疹结节型和浸润斑块型为主,而肿瘤样型少见。因CRDD临床缺乏特征性,极易漏诊或误诊,组织病理及免疫组化有助于鉴别诊断,有效提高CRDD的诊断率。CRDD具有自限性,但随访出现复发或新发,故应建议患者长期随访。Objective To analyze the clinical manifestations,histopathological and immunohistochemical features of cutaneous Rosai-Dorfman disease(CRDD).Methods A total of 40 patients with CRDD were collected,the clinical and histopathological manifestations,treatment and prognosis of different types of skin lesions were analyzed.Results Among the 40 patients,25 cases(62.50%)were female and 15 cases(37.50%)were male.There were 26 cases with single lesion(65.00%)and 14 cases were multiple lesions(35.00%).There were 48 tissue sections,32 patients underwent single histopathological examination,8 patients underwent 2 histopathological examination.The 48 lesions showed 25 papular nodular lesions(52.08%),18 infiltrating plaques(37.50%)and 5 tumor-like lesions(10.42%).Among the 40 patients,34 cases(85.00%)were followed up,and 6 cases(15.00%)were lost to follow-up,with a follow-up period of 6~66 months.Twenty-two patients were treated,12 patients were not treated.In two cases,new skin lesions appeared,but the original skin lesions became flat and smaller than before.Histopathology showed the whole dermal layer and subcutaneous tissue were alternately stained with light and dark.There were different numbers of large tissue cells.Phagocytosis of lymphocytes,plasma cells and neutrophils was observed in the tissue cells,increased number of blood vessels,collagen fibrosis and lymphatic follicles can be seen.Immunohistochemistry showed the cell positive for S100 and CD68,while negative for CD1a.Conclusion The clinical manifestations of CRDD are mainly papule nodular type and infiltrating plaque type,while tumor type is rare.Histopathology and immunohistochemistry are helpful to differential diagnosis and improve the diagnostic rate of CRDD.

关 键 词：ROSAI-DORFMAN病 临床表现 组织病理 伸入运动 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]