中华皮肤科杂志 ›› 2022, Vol. 55 ›› Issue (11): 1019-1020.doi: 10.35541/cjd.20220324

• 专家视角 • 上一篇    下一篇

假性血管性水肿：一种新的皮肌炎皮疹

杨骥    徐欣植    李明   

  1. 复旦大学附属中山医院皮肤科，上海  200032
  • 收稿日期:2022-05-09 修回日期:2022-05-31 发布日期:2022-11-03
  • 通讯作者: 杨骥 E-mail:yang.ji@zs-hospital.sh.cn
  • 基金资助:
    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

Pseudoangioedema: a new type of skin lesions of dermatomyositis

Yang Ji, Xu Xinzhi, Li Ming   

  1. Department of Dermatology, Zhongshan Hospital, Fudan University, Shanghai 200032, China
  • Received:2022-05-09 Revised:2022-05-31 Published:2022-11-03
  • Contact: Yang Ji E-mail:yang.ji@zs-hospital.sh.cn
  • Supported by:
    National Natural Science Foundation of China （82073436）; Medical Guide Project from Science and Technology Commission of Shanghai Municipality （CN） （20ZR1411500, 19411962500）; Special Clinical Research Project of Shanghai Municipal Health Commission （202140271）; Outstanding Youth Medical Talents of Shanghai "Rising Stars of Medical Talent" Youth Development Program; Shanghai Talent Development Fund; Zhuoxue Plan of Fudan University

PDF

416

摘要： 【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。

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

Abstract: 【Abstract】 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 1γ antibodies, 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.

Key words: Dermatomyositis, Pseudoangioedema, Transcriptional intermediary factor 1 gamma, Melanoma differentiation-associated gene 5