种痘样水疱病样淋巴组织增生性疾病2例诊治分析并文献复习  

Diagnosis and treatment of two cases of hydroa vacciniforme-like lymphoproliferative disorder and literature review

作　　者：黄慧 胡云峰[1] 刘赛君[1] 邓列华[1] HUANG Hui;HU Yunfeng;LIU Saijun;DENG Liehua(Department of Dermatology, the First Affiliated Hospital, Jinan University/Jinan University Institute of Dermatology, Guangzhou 510632,China;The Second Clinical College, Guangzhou University of Chinese Medicine, Guangzhou 510006, China)

机构地区：[1]暨南大学附属第一医院皮肤科/暨南大学皮肤病研究所,广东广州510632 [2]广州中医药大学第二临床医学院,广东广州510006

出　　处：《暨南大学学报（自然科学与医学版）》2020年第4期336-342,共7页Journal of Jinan University(Natural Science & Medicine Edition)

基　　金：广东省医学科学技术研究基金(A2016594)。

摘　　要：目的:探讨种痘样水疱病样淋巴组织增生性疾病(HV-LPD)的临床特征、转归与预后.方法:分析2例HV-LPD典型患者的皮疹特点、伴发症状、实验室检查、皮损组织病理及免疫组化、治疗预后等资料,再结合文献复习进行归纳总结.结果:2例女性患者青春期发病,以面部红斑、丘疹、丘疱疹为共同特征,血液EB病毒(EBV)DNA定量均明显高于正常值.皮损组织病理:均见表皮下水疱形成,真皮或皮下组织间血管及附属器周围弥漫淋巴样细胞,有异型性.免疫组化抗体结果显示:病例1表达CD3、CD4、CD8,Ki67(+)约10%;病例2表达CD7、CD56,Ki67(+)约占80%;EBV、GrB、TIA-1均表达.病例1给予干扰素、盐酸伐昔洛韦、醋酸泼尼松片治疗后病情缓解.病例2给予CHOP化疗、局部放疗等治疗,半年后死亡.复习近年国内外相关文献,发现HV-LPD临床表现具有一定特异性,并与其愈后转归密切相关,本研究总结的HV-LPD临床症状分值表,分数越高代表病情越严重,预后越差,可用于临床直观清晰地分析患者病情.结论:HV-LPD临床表现广泛且呈慢性发病过程,可进展为T/NK细胞淋巴瘤,HV-LPD临床症状分值表可协助分析病情严重程度.Objective:To explore the clinical features,outcome and prognosis of hydroa vacciniforme-like lymphoproliferative disorder(HV-LPD).Methods:This study analyzed the rash features,accompanying symptoms results,laboratory results,histopathology and immunohistochemistry,treatment prognosis of two cases with typical HV-LPD,and summarize the relevant literature.Results:The two cases were adolescent onset and were characterized by facial erythema,pimples and papule vesicle.Quantitative Analysis of Plasma Epstein-Barr Virus DNA were significantly higher than normal values.Histopathology of skin lesions showed the formation of subepidermal blisters,diffuse lymphoid cells around blood vessels and appendages in the dermis or subcutaneous tissue,and atypical.Immunohistochemistry of case 1 expressed CD3,CD4 and CD8,Ki67(+)was about 10%;while case 2 expressed CD7 and CD56,Ki67(+)was about 80%;EBV,GrB and TIA-1 all expressed.Case 1 was treated with interferon,valacyclovir hydrochloride,prednisone acetate,and the condition eased.Case 2 was given CHOP chemotherapy,local radiotherapy etc.And died six months later.This study reviewed the relevant papers at home and abroad in recent years,and found that the clinical manifestations of HV-LPD were specific and closely related to their prognosis.The HV-LPD clinical features score table summarized in this study indicates that a higher score indicates a more serious condition and a worse prognosis,which can be used to analyze patient's condition intuitively and clearly.Conclusion:HV-LPD has a wide range of clinical manifestations and a chronic pathogenesis,and may develop into T/NK cell lymphoma.HV-LPD clinical features score table can help to analyze the condition.

关 键 词：种痘样水疱病样淋巴组织增生性疾病 EB病毒(EBV) T/NK细胞淋巴瘤 

分 类 号：R751[医药卫生—皮肤病学与性病学]