EB病毒感染相关的皮肤淋巴增殖性疾病二例

Epstein-Barr virus-associated cutaneous lymphoproliferative disease： two case report

目的 报道EB病毒感染相关的皮肤淋巴增殖性疾病2例及其与慢性活动性EB病毒感染的关系.方法 分析2例皮肤淋巴增殖性疾病患者的临床资料、实验室检查、治疗和转归.结果 2例患者均有间断性发热,淋巴结肿大,反复发作的丘疹、丘疱疹、坏死、痘疮样瘢痕,皮疹分布于曝光和非曝光部位.皮损组织病理示真皮内淋巴样细胞浸润,细胞形态异形,侵犯血管周围及部分皮下组织.免疫组化显示浸润细胞以CD8＋细胞为主;T细胞受体γ基因（TCRγ基因）阴性;EB病毒原位杂交阳性.外周血EB病毒DNA拷贝数高于正常.糖皮质激素治疗后病情缓解.结论 2例皮肤淋巴增殖性疾病的生物学行为呈慢性惰性经过,与慢性活动性EB病毒感染密切相关.

Objective To report 2 cases of Epstein-Barr virus-associated cutaneous lymphoproliferative disease （LPD） and to evaluate their relationship with chronic active Epstein-Barr virus （CAEBV） infection.Methods The clinical data on, laboratory examination findings in, treatment and therapeutic response of 2cases of LPD were analyzed. Results Both the patients had chronic intermittent fever, lymphadenopathy, recurrent lesions including papules, papulovesicles, necrosis and variola-like scar in light-exposed and unexposed areas. Pathologically, there was a dermal infiltrate of pleomorphic lymphoid cells with the involvement of perivascular area and some subcutaneous tissue. Immunohistochemical staining showed that most of the infiltrating lymphoid cells were positive for CD8, PCR revealed no TCR-γ gene rearrangement, and in situ hybridization for EBV was positive. The copy of EBV DNA was above the normal range in the peripheral blood from both patients. Clinical status was improved after glucocorticoid treatment. Conclusion The biologic behavior of LPD appears to be a chronic and indolent course and is closely associated with CAEBV.