种痘水疱病样皮肤T细胞淋巴瘤1例.pdfVIP

· 366· 中国皮肤性病学杂志2008年6月第22卷第6期 Chin J Derm Venereol，June．2008Vo1．22，N0．6 ， · 病例报告 · 种痘水疱病样皮肤T细胞淋巴瘤1例 祁怀山，郭一峰，陈洁，余红，姚志荣 [摘 要】 患儿女，5岁。面部、手背反复发作红斑、水疱4年，皮疹消退后遗留下痘疮样萎缩性瘢痕。血清抗EBV IgG(+)、EBV IgM(一)。皮损组织病理示：真皮全层及皮下脂肪弥漫性淋巴细胞浸润，伴少量嗜酸性粒细胞，可见核分裂 象。免疫组化示LCA(+)、UCHL 1(+)、CD3(+)、CD5(+)、CD10(+)、CD99(弱+)。基因重排检测示：TCR一 基因 JVI(+)、JVII(弱+)。诊断：种痘水疱病样皮肤T细胞淋巴瘤。明确诊断后转至本院血液科，予CHOP方案化疗，现随访 中。 【关键词】 种痘样水疱病；T细胞淋巴瘤；Epstein—Barr病毒 [中图分类号]R 739．5 [文献标识码] B [文章编号] 1001—7089(2008)06—0366—03 Hyroa Vacciniforme—like Cutaneous T Cell Lymphoma：A Case Report QI Huai—shan，CHEN Jie，Yu Hong，et al (Department ofDermatology，Xinhua Hospital Affiliated to Medical School，Shanghai Jiaotong University，Shanghai 200092，Chi一 ， ) Abstract：Objective A 5——year—-old girl presented with erythemas and papules on face and extremities as well as fever and itching for 4 years．When the lesions dissipated，pitted scars left．The viral serological diagnosis showed EBV IgG positivity，but EBV IgM negativity．Histopathology of the skin showed diffuse and dense infiltrate of lymphoid cells with anasplastic nuclei in the dermis as well as the subcutis．The heteromorphic cells were positive for LCA，UCHL1，CD3，CD5，CD10 and CD99．Detection of gene rearrangement show gene of TCR一 JVI positivity and JVI1 weakly positivity．A diagnosis of Hydroa vacciniforme—like cutaneous T cell lymphoma was made． Key words： Hydroa vacciniforme；Cutaneous T cell lymphoma；Epstein—Barr virus 种痘水疱病样皮肤T细胞淋巴瘤(Hydroa vacciniforme—like 23 400IU／mL(正常值100IU／mL)。抗核抗体(ANA)、抗可浸 cutaneous T cell lymphoma)是一种罕见的皮肤T细胞淋巴瘤。该 出核抗原(ENA)、抗dsDNA均为阴性。EBV抗体一IgG(+)， 病多见于儿童，与Epstein—Barr病毒(EBV)感染相关…，皮损易 EBV—IgM(一)。胸部cT示两腋下多发淋巴结影，胸腺形态无 与种痘样水疱病相混淆。本科近期诊治1例，