种痘水疱病样淋巴组织增生性疾病并发噬血细胞综合征  

Hydroa vacciniforme-like lymphoproliferative disorder complicated with hemophagocytic syndrome:a case report

作　　者：丁书红 李敏 杨苏[1] 苏婷[2] 鲁严[2] 俞海国[3] 杨潇 DING Shu-hong;LI Min;YANG Su;SU Ting;LU Yan;YU Hai-guo;YANG Xiao(Department of Dermatology,Children’s Hospital of Nanjing Medical University,Nanjing 210008,China)

机构地区：[1]南京医科大学附属儿童医院皮肤科,江苏南京210008 [2]南京医科大学第一附属医院皮肤科,江苏南京210009 [3]南京医科大学附属儿童医院风湿科,江苏南京210008

出　　处：《临床皮肤科杂志》2022年第6期355-357,共3页Journal of Clinical Dermatology

摘　　要：报告1例种痘水疱病样淋巴组织增生性疾病并发噬血细胞综合征。患儿男,13岁。因面部和四肢反复丘疹、丘疱疹、水疱及溃疡伴发热8年,加重2周入院。皮肤科检查:面部及四肢弥漫性肿胀,其上泛发暗红斑、丘疹、丘疱疹及溃疡,溃疡基底部覆有脓苔,其间可见大量痘疮样瘢痕;部分手指肌腱外露。实验室检查:血清Epstein-Barr病毒(EBV)拷贝数明显升高。左下肢皮损组织病理检查:大部分表、真皮坏死结痂,痂内及痂下方血管闭塞,管周淋巴细胞浸润,并可见个别病理性核分裂象。诊断:种痘水疱病样淋巴组织增生性疾病。入院后患儿存在持续发热,肝、脾大以及其他血液指标异常,提示并发嗜血细胞综合征。A case of hydroa vacciniforme-like lymphoproliferative disorder complicated with hemophagocytic syndrome is reported. A13-year-old male presented with recurrent rashes on the face and extremities accompanied by fever for 8 years, which was worsened for 2 weeks. Physical examination showed diffuse edema on his face and limbs with erythema, papule, papulovesicle, ulcer, and varicelliform scars. Some of the finger tendons were exposed. The EBV copy number in the blood was significantly increased. Histopathological examination showed most epidermis and dermis of the lesions were necrotic and crusted, the blood vessels in and below the crust were occluded, lymphocytes were infiltrated around the tube, and individual pathological mitoses were seen. The patients was preliminarily diagnosed with hydroa vacciniforme-like lymphoproliferative disorder. Based on his persistent fever, enlarged liver and spleen, as well as other abnormal serum indicators, the final diagnosis was hydroa vacciniforme-like lymphoproliferative disorder complicated with hemophagocytic syndrome.

关 键 词：种痘水疱病样淋巴组织增生性疾病 噬血细胞综合征 儿童 

分 类 号：R733.1[医药卫生—肿瘤]