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Dermatomyositis is considered a type of inflammatory myopathy. [1] Diagnosis is typically based on some combination of symptoms, blood tests, electromyography, and muscle biopsies. [2] Eighty percent of adults [5] and sixty percent of children with juvenile dermatomyositis have a myositis-specific antibody (MSA). [6]
Around 3.2 children per million per year are diagnosed with DM (termed juvenile dermatomyositis), with an average age of onset of seven years. Diagnosis of adult DM commonly occurs between 30 and 50 years of age. PM is an adult disease, usually emerging after the age of twenty.
The three main types of idiopathic myositis (known as inflammatory myopathies) that typically test positive for autoantibodies are dermatomyositis, polymyositis, and inclusion body myositis. [4] Other autoimmune diseases, such as systemic lupus erythematosus, can also cause myositis-like symptoms. [4]
Dermatomyositis produces muscle weakness and skin changes. The skin rash is reddish and most commonly occurs on the face, especially around the eyes, and over the knuckles and elbows. Ragged nail folds with visible capillaries can be present.
Gottron's sign is a pathognomonic cutaneous manifestation associated with dermatomyositis (DM), which is an inflammatory disorder affecting the skin and muscles. [1] The primary lesion of dermatomyositis appears as a violaceous, macular erythema with a symmetric distribution, which may progress and become poikilodermatous (atrophic with telangiectasia and pigmentary changes) and indurated (as ...
Polymyositis and the associated inflammatory myopathies have an associated increased risk of cancer. [3] The features they found associated with an increased risk of cancer were older age, age greater than 45, male sex, difficulty swallowing, death of skin cells, cutaneous vasculitis, rapid onset of myositis (<4 weeks), elevated creatine kinase, higher erythrocyte sedimentation rate and higher ...
Gowers's sign is classically seen in Duchenne muscular dystrophy where it is mostly evident at 4–6 years, but also presents itself in centronuclear myopathy, myotonic dystrophy and various other conditions associated with proximal muscle weakness, including Becker muscular dystrophy, dermatomyositis and Pompe disease.
3.3 per 100,000 (adults), 50 per 100,000 (children) [90] Thrombotic thrombocytopenic purpura: ADAMTS13 autoantibodies Confirmed 1-2 per million [91] Antiphospholipid syndrome: Antiphospholipid antibodies Confirmed 40-50 per 100,000 [92] Paroxysmal nocturnal hemoglobinuria: None specific, mutation causes self-cells to become susceptible to ...