中华皮肤科杂志

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牛痘样水疱病样皮肤淋巴瘤与慢性活动性EB病毒感染的关系

徐子刚1, 周春菊2, 肖媛媛1, 马琳1, 赵佩云1   

  1. 1. 首都医科大学附属北京儿童医院皮肤科 北京 100045;
    2. 首都医科大学附属北京儿童医院病理科 北京 100045
  • 收稿日期:2007-01-23 出版日期:2007-12-15 发布日期:2007-12-15

Relationship between hydroa vacciniforme-like cutaneous lymphoma and chronic active Epstein-Barr virus infection

XU Zi-gang1, ZHOU Chun-ju2, XIAO Yuan-yuan1, MA Lin1, ZHAO Pei-Yun1   

  1. Department of Dermatology,Beijing Children's Hospital, Capital Medical University, Beijing 100045, China
  • Received:2007-01-23 Online:2007-12-15 Published:2007-12-15

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摘要： 目的 报道6例牛痘样水疱病样皮肤淋巴瘤,并研究其与慢性活动性EB病毒感染的关系.方法 临床病理分析、皮损免疫组织化学染色、血清学分析、EB病毒编码RNA原位杂交、外周血EB病毒DNA测定.结果 6例患者皮损均为反复发作的丘疹、丘疱疹、坏死、痘疮样瘢痕,其中4例还伴有程度不同的颜面、手足水肿.所有患儿均有长期间断发热等症状.皮损病理可见表皮多房性水疱,真皮全层大量淋巴细胞浸润,细胞形态异形,可见病理分裂象.4例皮损病理免疫组化染色,可见大量CD56阳性细胞,散在的CD3和CD45RO阳性细胞,T细胞内抗原-1和粒酶B染色阳性,诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤;2例组化染色CD3和CD45RO阳性,CD56阴性,诊断为牛痘样水疱病样皮肤T细胞淋巴瘤.6例皮损均可见EB病毒编码RNA原位杂交阳性肿瘤细胞,血清学检查EB病毒衣壳抗原IgG抗体滴度升高,其中2例滴度为1:5120,2例为1:2560,2例为1:1280;2例患者外周血EB病毒DNA拷贝数高于正常.6例患儿均证实患有慢性活动性EB病毒感染.结论 牛痘样水疱病样皮肤淋巴瘤主要表现为颜面手足肿胀、水疱、痘疮样瘢痕,病理表现主要为真皮异形淋巴细胞浸润和血管中心坏死,免疫表型以NK/T型多见.慢性活动性EB病毒感染与该型淋巴瘤发病密切相关.

关键词: 淋巴瘤, 杀伤细胞,天然, 疱疹病毒4型,人, 爱泼斯坦巴尔病毒感染

Abstract: Objective To report 6 cases of hydroa vacciniforme-like cutaneous lymphoma,and to investigate the relationship between this disorder and Epstein-Barr virus(EBV)infection.Methods Pathological and immunohistochemical examinations were performed in the biopsy specimens obtained from all 6 patients. Skin lesions were subjected to EBV encoded RNA(EBER)detection by in situ hybridization.Serological assay and quantification of EBV DNA were performed.Results All the 6 patients had recurrent papules, papulovesicles,necrosis and variola-like scar with chronic intermittent fever;four of the patients also presented with edema of the face,hands and feet.Pathologically,there were multilocular vesicles in the epidermis,and large numbers of infiltrating lymphocytes through the dermis.The cells were atypical with mitotic figures. Immunohistochemical staining of the lesions of 4 patients showed large quantities of cells expressing CD56, scattered cells expressing CD3 and CD45RO,and cells expressing grazyme B and T cell intracellular antigen-1 (TIA-1);a diagnosis of hydroa vacciniforme-like cutaneous NK/T lymphoma was made in these 4 cases. In the lesions of another 2 patients,the cells expressing CD3 and CD45RO,but not CD56,were observed; the diagnosis of hydroa vacciniforme-like cutaneous T-cell lymphoma was made in them.EBER was detected in the tumor cells of all the 6 patients.The IgG titers of anti-Epstein-Barr viral capsid antigen increased in all patients(1:5120 in 2 cases,1:2560 in 2 cases,1:1280 in 2 cases).The copies of EBV DNA were increased in the peripheral blood of both the two detected cases.A chronic active EBV infection was confirmed in all patients.Conclusions Hydroa vacciniforme-like cutaneous lymphoma is clinically characterized by edema of face,hands and feet,vesicular eruptions and variola like scars;histologically,it is characterized by infiltrates of atypical cells consistent with lymphoma,and necrosis in the center of vessels.NK/T is the primary immunophenotype of this disease.There is a close association between chronic active EBV infection and hydroa vacciniforme-like cutaneous lymphoma.

Key words: Lymphoma, Killer cells, natural, Herpesvirus 4,human, Epstein-Barr virus infections