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Concept
Dermatomyositis
Summary
Dermatomyositis (DM) is a long-term inflammatory disorder which affects the skin and the muscles. Its symptoms are generally a skin rash and worsening muscle weakness over time. These may occur suddenly or develop over months. Other symptoms may include weight loss, fever, lung inflammation, or light sensitivity. Complications may include calcium deposits in muscles or skin.
The cause is unknown. Theories include that it is an autoimmune disease or a result of a viral infection. Dermatomyositis may develop as a paraneoplastic syndrome associated with several forms of malignancy. It is a type of inflammatory myopathy. Diagnosis is typically based on some combination of symptoms, blood tests, electromyography, and muscle biopsies.
Eighty percent of adults with adult-onset dermatomyositis have a myositis-specific antibody (MSA).
Sixty percent of children with juvenile dermatomyositis have a myositis-specific antibody (MSA).
Although no cure for the condition is known, treatments generally improve symptoms. Treatments may include medication, physical therapy, exercise, heat therapy, orthotics, and assistive devices, and rest. Medications in the corticosteroids family are typically used with other agents such as methotrexate or azathioprine recommended if steroids are not working well. Intravenous immunoglobulin may also improve outcomes. Most people improve with treatment and in some, the condition resolves completely.
About one per 100,000 people per year are newly affected. The condition usually occurs in those in their 40s and 50s with women being affected more often than men. People of any age, however, may be affected. The condition was first described in the 1800s.
The main symptoms include several kinds of skin rash along with muscle weakness in both upper arms or thighs.
One form the rashes take is called "heliotrope" (a purplish color) or lilac, but may also be red. It can occur around the eyes along with swelling, but also occurs on the upper chest or back what is called the "shawl" (around the neck) or "V-sign" above the breasts and may also occur on the face, upper arms, thighs, or hands.
Official source
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Ontological neighbourhood
Related publications (2)
Assessment of idiopathic inflammatory myopathy using a deep learning method for muscle T2 mapping segmentation
Qian Wang, Tobias Kober, Tom Hilbert, Yao Zhang, Yubo Zhao
Objective To investigate the utility of an automatic deep learning (DL) method for segmentation of T2 maps in patients with idiopathic inflammatory myopathy (IIM) against healthy controls, and also the association of quantitative T2 values in patients with ...
SPRINGER2022Wearable exoskeletons to support ambulation in people with neuromuscular diseases, design rules and control
Neuromuscular diseases are degenerative and, thus far, incurable disorders that lead to large muscle wasting. They result in constant deterioration of activities of daily living and in particular of ambulation. Some common types include Duchenne muscular d ...
EPFL2019Related concepts (17)
Polymyositis
Polymyositis (PM) is a type of chronic inflammation of the muscles (inflammatory myopathy) related to dermatomyositis and inclusion body myositis. Its name means 'inflammation of many muscles' (poly- + myos- + -itis). The inflammation of polymyositis is mainly found in the endomysial layer of skeletal muscle, whereas dermatomyositis is characterized primarily by inflammation of the perimysial layer of skeletal muscles. The hallmark of polymyositis is weakness and/or loss of muscle mass in the proximal musculature, as well as flexion of the neck and torso.
Inclusion body myositis
Inclusion body myositis (IBM) (maIou'saItIs) (sometimes called sporadic inclusion body myositis, sIBM) is the most common inflammatory muscle disease in older adults. The disease is characterized by slowly progressive weakness and wasting of both proximal muscles (located on or close to the torso) and distal muscles (close to hands or feet), most apparent in the finger flexors and knee extensors. IBM is often confused with an entirely different class of diseases, called hereditary inclusion body myopathies (hIBM).
Scleroderma
Scleroderma is a group of autoimmune diseases that may result in changes to the skin, blood vessels, muscles, and internal organs. The disease can be either localized to the skin or involve other organs, as well. Symptoms may include areas of thickened skin, stiffness, feeling tired, and poor blood flow to the fingers or toes with cold exposure. One form of the condition, known as CREST syndrome, classically results in calcium deposits, Raynaud's syndrome, esophageal problems, thickening of the skin of the fingers and toes, and areas of small, dilated blood vessels.