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The purpose of this article is to review the etiopathogenesis of dermatomyositis, including the predisposing factors, triggers, inflammatory cells, pathways and target antigens associated with dermatomyositis.During the last few years, we have made considerable progress in unveiling the etiopathogenesis of dermatomyositis. In the first place, we have defined genes within the major histocompatibility complex locus as the greatest genetic risk factor for the disease. Second, we have demonstrated that certain environmental factors, as well as tumors, may trigger certain dermatomyositis subtypes. Moreover, we have established the importance of the interferon pathway in dermatomyositis pathogenesis compared with other myositis subtypes. But probably, the most remarkable advance has been the discovery of multiple autoantibodies that define groups of patients with characteristic clinical features, prognosis and response to treatment.Dermatomyositis cause and pathogenesis have proven to be a complex and fascinating task for the scientific community and the last decade has been full of new findings on how the disease starts and how it causes damage to different organ systems. However, we have still more questions than answers in this topic, answers that will be critical to understanding autoimmunity and finding effective therapies to dermatomyositis.