Dermatomyositis

Dermatomyositis is an autoimmune inflammatory disease marked by characteristic skin eruptions and, in many cases, progressive weakness of muscles close to the trunk. It can also affect the lungs and other organs.

Type I interferon: A family of signaling proteins that coordinates antiviral defenses and can promote autoimmune inflammation. Excess interferon signaling in affected tissue is a prominent feature of dermatomyositis.
Gottron papules: Raised, often scaly red or violet lesions over the knuckles and other extensor joints. These hallmark lesions strongly support a dermatomyositis diagnosis.
Glucocorticoid: A steroid hormone or medication that suppresses inflammation and immune activity. Systemic glucocorticoids are commonly used initially for significant muscle inflammation.
Clinically amyopathic dermatomyositis: Dermatomyositis with characteristic skin disease but no clinically significant muscle weakness. It shows that classic skin disease can occur without the usual muscle presentation.
Complement system: A network of blood proteins that helps target pathogens and damaged cells for immune attack. Complement deposition in small vessels is implicated in dermatomyositis tissue injury.
Heliotrope rash: A violet or dusky rash around the eyelids, sometimes accompanied by eyelid swelling. This distinctive facial eruption is a classic cutaneous sign of dermatomyositis.
Intravenous immunoglobulin: A preparation of pooled antibodies given by vein to modify immune activity. It can improve muscle and skin disease when standard immunosuppressive treatment is insufficient.
Juvenile dermatomyositis: Dermatomyositis beginning in childhood, with characteristic skin and muscle inflammation. Childhood disease differs in common complications and is not typically linked to cancer.
Perifascicular atrophy: Muscle-fiber shrinkage concentrated at the edges of muscle fascicles, a characteristic biopsy finding in dermatomyositis. This pattern helps distinguish dermatomyositis-related muscle injury on biopsy.
Proximal muscle weakness: Reduced strength in muscles near the trunk, such as those used to climb stairs or lift the arms. This is the typical pattern of muscle impairment when dermatomyositis affects muscle.

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