Systemic dermatomyositis

Systemic dermatomyositis is a rare, immune-mediated idiopathic inflammatory myopathy characterized by widespread microvascular injury and multisystem involvement that affects both children and adults Beyond skeletal muscle and skin, the systemic disease frequently involves the pulmonary, gastrointestinal, and cardiovascular systems, presenting with complications such as interstitial lung disease, esophageal dysmotility, and arthritis, along with an elevated risk of underlying malignancy in adult...

Systemic dermatomyositis is a rare, immune-mediated idiopathic inflammatory myopathy characterized by widespread microvascular injury and multisystem involvement that affects both children and adults

It classically manifests with hallmark cutaneous findings—such as a heliotrope rash and Gottron papules—paired with symmetric proximal muscle weakness and inflammatory myositis Beyond skeletal muscle and skin, the systemic disease frequently involves the pulmonary, gastrointestinal, and cardiovascular systems, presenting with complications such as interstitial lung disease, esophageal dysmotility, and arthritis, along with an elevated risk of underlying malignancy in adult patients

It classically manifests with hallmark cutaneous findings—such as a heliotrope rash and Gottron papules—paired with symmetric proximal muscle weakness and inflammatory myositis

Beyond skeletal muscle and skin, the systemic disease frequently involves the pulmonary, gastrointestinal, and cardiovascular systems, presenting with complications such as interstitial lung disease, esophageal dysmotility, and arthritis, along with an elevated risk of underlying malignancy in adult patients

Pathophysiologically, it is driven by complement-mediated microangiopathy and robust type I interferon pathway activation, typically accompanied by distinct myositis-specific autoantibodies