种痘样水疱病样淋巴组织增生性疾病是一种特殊的皮肤淋巴细胞增殖性疾病,与EB病毒感染有直接的关系.该病在东亚地区,包括日本、中国等地报告较多.种痘样水疱病样淋巴瘤容易发生在儿童,表现为面部、手背等光暴露部位为主的丘疱疹,愈后常遗留萎缩性瘢痕.患者常并发有系统表现,如发热、淋巴结增大,肝脾增大等,部分患者预后不良.种痘样水疱病样淋巴组织增生性疾病属于谱系性疾病,二者之间常无法严格区分,临床常见到种痘样水疱病转化为种痘样水疱病样淋巴瘤的病例.该文简要综述了种痘样水疱病样淋巴组织增生性疾病的临床和组织病理特点、发病机制以及治疗进展.%Hydroavacciniforme-like lymphoproliferative disorder is a specific cutaneous lymphatic proliferation associated with EB virus infection. The disease is usually reported in east Asian countries including Japan and China. The disease usually developed on children and presented as papulovesicles on sun exposed areas such as face and dorsum of hands, which usually heals with atrophic scar. The patients are usually complicated with systemic symptoms such as fever, lymphoadenopathy and hepatosplenomegaly, and some patients show unfavourable prognosis. In this review we briefly introduced the clinical and histopathological characters, the pathogenesis and the current treatments of hydroavacciniforme-like lymphoproliferative disorder.
展开▼
