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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。
Objective To report 6 cases of vaccinia-like blister-like cutaneous lymphoma and study its relationship with chronic active Epstein-Barr virus infection. Methods Clinicopathological analysis, immunohistochemical staining, serological analysis, Epstein - Barr virus coding RNA in situ hybridization, peripheral blood Epstein - Barr virus DNA determination. Results The skin lesions of 6 patients were recurrent papules, herpes pylorus, necrosis, and acne scars. Four of them were accompanied by different degrees of face, hand and foot edema. All children have long-term fever and other intermittent symptoms. Pathological lesions showed multiple epidermal blisters, dermis a large number of full-thickness lymphocytes infiltration, morphological abnormal cell shape, showing pathological mitosis. Immunohistochemical staining of 4 lesions showed that a large number of CD56 positive cells, scattered CD3 and CD45RO positive cells, positive T-cell antigen-1 and granzyme B staining were diagnosed as vaccinia-like skin-like skin NK / T cell lymph Tumor; 2 cases of CD3 and CD45RO positive staining, CD56 negative, diagnosed as vaccinia-like blister-like cutaneous T-cell lymphoma. 6 cases of lesions were visible Epstein-Barr virus RNA in situ hybridization positive tumor cells, serological examination of EB virus capsid antigen IgG antibody titers, including two cases of titer 1: 5120, 2 cases of 1: 2560,2 For example 1: 1280; 2 cases of peripheral blood Epstein-Barr virus DNA copy number higher than normal. All 6 patients were confirmed to have chronic active Epstein-Barr virus infection. Conclusions Vaccinia-like blister-like cutaneous lymphoma mainly manifested as hand, foot and foot swelling, blisters and acne-like scars. The main pathological manifestations were dermal abnormal lymphocyte infiltration and vascular center necrosis. The immunophenotype was more common in NK / T type. Chronic active Epstein-Barr virus infection is closely related to the onset of this type of lymphoma.