Abstract: 【Abstract】 Dermatomyositis with malignancy is a subtype of dermatomyositis, and the concurrence of malignancy is a major factor for poor prognosis of dermatomyositis. Malignancy usually occurs within 1 year before or after the diagnosis of dermatomyositis. Compared with classic dermatomyositis, dermatomyositis with malignancy mostly occurs in patients aged ＞50 years, and is typically characterized by the heliotrope sign, skin necrosis and presence of myositis-specific antibodies such as anti-transcriptional intermediary factor-1γ and anti-nuclear matrix protein-2 antibodies, etc. Thorough screening for malignancy or close monitoring and follow-up are necessary in patients with dermatomyositis.

Key words: Dermatomyositis, Neoplasms, Autoantibodies, Diagnosis, Prognosis