假性血管性水肿:一种新的皮肌炎皮疹  

Pseudoangioedema: a new type of skin lesions of dermatomyositis

作　　者：杨骥 徐欣植 李明 Yang Ji;Xu Xinzhi;Li Ming(Department of Dermatology,Zhongshan Hospital,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属中山医院皮肤科,上海200032

出　　处：《中华皮肤科杂志》2022年第11期1019-1020,共2页Chinese Journal of Dermatology

基　　金：国家自然科学基金(82073436);上海市科研计划项目(20ZR1411500、19411962500);上海市卫生健康委员会临床研究专项(202140271);上海市"医苑新星"青年医学人才培养计划项目;上海市人才发展基金;复旦大学"卓越2025"-卓学优秀人才层次。

摘　　要：介绍一种新的皮肌炎皮疹类型——假性血管性水肿,其特点为患者面部、口唇、四肢为主出现局限性或弥漫性水肿,非凹陷性,可伴或不伴红斑,皮疹无明显瘙痒。大部分伴假性血管性水肿的皮肌炎患者抗转录中介因子1γ抗体阳性,会出现严重肌肉损伤,肌酶异常增高,以及难治性吞咽困难;少部分患者抗黑素瘤分化相关基因5抗体阳性,此类患者均有肺部累及。因此,皮肌炎患者出现假性血管性水肿时需要高度重视,抗体筛查有助于患者早期诊断和早期差异化治疗,改善患者的症状体征,提高患者的生存率。Pseudoangioedema,a new type of skin lesions of dermatomyositis,is characterized by localized or diffused,non-pitting,non-pruritic edema on the face,lips,and limbs,with or without erythema.Most dermatomyositis patients with pseudoangioedema are positive for anti-transcriptional intermediary factor 1γantibodies,and experience severe muscle injuries,increase of serum creatine kinase levels,and refractory dysphagia.A small number of dermatomyositis patients with pseudoangioedema are positive for anti-melanoma differentiation-associated gene 5 antibodies,and have lung involvement.Therefore,high attention should be paid to the occurrence of pseudoangioedema in patients with dermatomyositis,and the screening of specific antibodies can facilitate early diagnosis and differential treatment,as well as improvement in symptoms and survival rates.

关 键 词：皮肌炎 假性血管性水肿 转录中介因子1γ 黑素瘤分化相关基因5 

分 类 号：R593.26[医药卫生—内科学]