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