摘要
目的总结1例牛痘样水疱病样皮肤淋巴瘤的临床特点和组织病理学特征,旨在提高对这一疾病的认识。方法收集患者的临床资料,观察其组织学形态并行免疫组化和EB病毒原位杂交检测。结果患儿女性,7岁。2年内面部、四肢及躯干部反复出现红斑、水疱,疱破后结痂,遗留痘疮样瘢痕,伴长期间断发热。皮肤活检:表皮内见多房水疱,水疱内、真皮层及皮下脂肪组织可见中等大小异型淋巴细胞弥漫浸润,部分围绕皮肤附属器及血管呈小叶状分布,伴血管中心性浸润和坏死。肿瘤细胞间穿插分布少量嗜酸性粒细胞和组织细胞。免疫组化肿瘤细胞CD3、CD45RO、CD8、TIA-1、穿孔素、粒酶B和LMP-1(+),而CD56(-);原位杂交EBER(+)。结论牛痘样水疱病样皮肤淋巴瘤是好发于儿童的罕见皮肤淋巴瘤,诊断及鉴别诊断需结合临床皮疹特点、组织病理学形态、免疫组化及原位杂交综合分析。
Objective To investigate the clinical and pathological features,diagnosis and differential diagnosis of hydroa vacciniforme-like cutaneous lymphoma.Methods Clinical data of one patient was analyzed,and the morphological features were observed by light microscopy,immunohistochemistry,as well as in situ hybridization for Epstein-Barr virus（EBV）.Results A 7-year-old girl patient presented with two years symptoms of recurrent papules,papulovesicle,necrosis and variola-like scars on the face,extremities and trunk,accompanying with chronic intermittent fever.Pathologically,there were multilocular vesicles in the epidermis.Large quantities of medium-sized atypical lymphoid cells infiltrated from the epidermis to subcutaneous tissues around skin appendages and blood vessels in a lobular fashion,showing necrosis,angiocentricity and angioinvasion.Eosinophils and histiocyte distributed scatteredly among the tumor cells.Immunohistochemical staining showed tumor cells were positive for CD3,CD45RO,CD8,TIA-1（T cell intracellular antigen-1）,perforin,granzyme B and LMP1,and negative for CD56.Epstein-Barr virus-encoded RNA（EBER） was positive.Conclusions Hydroa vacciniforme-like cutaneous lymphoma is a rare EBV-associated tumor that affects children.The diagnosis and diffenertial diagnosis rely mainly on the comprehensive analysis of special skin lesions,histopathological and immunohistochemical findings,as well as EBV detection with in situ hybridization.
出处
《诊断病理学杂志》
CSCD
北大核心
2013年第8期487-490,共4页
Chinese Journal of Diagnostic Pathology
