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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.
Autoimmune - Autoimmune disease is an abnormal immune response to specific body protein or other biomolecular target, such as one of the muscles. 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]
Once a diagnosis of JDMS is made, the treatment is often a 3-day course of Intravenous ("pulse") steroids (methylprednisolone, Solu-Medrol), followed by a high dose of oral prednisone (usually 1–2 mg/kg of body weight) for several weeks. This action usually brings the disease under control, lowering most lab tests to or near normal values.
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.
Myopathy means muscle disease (Greek : myo- muscle + patheia -pathy : suffering). This meaning implies that the primary defect is within the muscle, as opposed to the nerves (" neuropathies " or " neurogenic " disorders) or elsewhere (e.g., the brain).
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 ...
Guillain-Barré syndrome (GBS) is the main consideration in the differential diagnosis. It needs to be quickly excluded as early intervention in GBS is indicated. Other conditions under possible consideration are dermatomyositis, muscular dystrophy, juvenile idiopathic arthritis, transient synovitis of the hip, osteomyelitis, and myalgia. [1]