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It may also affect other organs, such as the heart and lungs. Dermatomyositis belongs to a rare group of conditions called inflammatory myopathies. These conditions share certain symptoms, including:skin rashesmuscle weaknesstendernessswelling The symptoms vary widely from person to person, but it is generally possible to manage them with treatment. In this article, we discuss dermatomyositis in detail, including the causes, risk factors, symptoms, diagnosis, treatment, and potential complications. We also look at the outlook for people with dermatomyositis and explain how this disease differs from other health conditions. What is this condition Dermatomyositis is a rare acquired muscle condition, with an incidence of 9.63 per 1 million people. The condition affects females twice as often as males. All inflammatory myopathies affect the muscles. Dermatomyositis causes weakness of the proximal muscles, which are those near the trunk. It may also affect other parts of the body, including the:skingastrointestinal systemlungsheart Signs and symptoms The symptoms of dermatomyositis may be acute, appearing suddenly, or insidious, developing gradually. Muscle weakness is the most common symptom. People may also experience:sorenesstendernessstiffnessmuscle wasting, which is called atrophy It often affects muscles in the:neckhipsthighsshouldersupper arms The symptoms may lead to difficulty carrying out certain activities, including:combing the hairlifting the armsclimbing stepsdressinggetting up from the floorraising the head from the pillow Other muscle groups The condition may also affect other muscle groups, such as the throat, tongue, chest, and heart. This can lead to:difficulty swallowing, known as dysphagiadifficulty speaking, or dysphoniabreathing difficultiesarrhythmia, which refers to abnormal heart rhythmsheart muscle weakness, or cardiomyopathy Skin changes In 40% of people, skin changes may precede muscle weakness. The two hallmark features of the condition are Gottron’s papules and a heliotrope rash. Gottron’s papules are reddish or violet bumps that appear on the surface of bony prominences such as the knuckles, elbows, and knees. A heliotrope rash is a patchy, uneven rash on the upper eyelids. Other possible skin changes include:reddish purple scaling patchespoikiloderma, in which the skin has finely mottled areas of hypopigmentation alongside:hyperpigmentationtelangiectasiaatrophya discolored scaly scalpnail bed changes Additional symptoms Other symptoms and associated conditions may include:calcinosis, which is the abnormal accumulation of calcium deposits in muscle and skin tissuesfluid accumulation, which is edema surrounding the eyespolyarthralgia, which is the term for pain in multiple joints low grade feverRaynaud s diseasepeptic ulcers Types Adult dermatomyositis typically occurs in people aged 40–50 years. There may also be a more frequent association between dermatomyositis and underlying cancers. Juvenile dermatomyositis is a form of dermatomyositis that affects children. Doctors usually diagnose it in children aged 5–15 years, and it is more common among white children. The symptoms associated with childhood dermatomyositis are the same as those of adult dermatomyositis, but the onset is more sudden, and the condition often presents with skin changes before muscle weakness. Widespread blood vessel inflammation, called vasculitis, and calcinosis — large calcium deposits under the skin — occur more frequently in children than in adults. Those affected may also develop a tiptoe gait secondary to ankle stiffness. However, unlike in the adult form, children with the condition are not at risk of cancer. Dermatomyositis sine myositis, which doctors also call amyopathic dermatomyositis, is another form of dermatomyositis. It presents with the typical skin lesions of dermatomyositis but without the associated muscle abnormalities. Causes and risk factors The cause of dermatomyositis remains unknown, but experts believe that a combination of factors plays a role, including:geneticenvironmentalimmune system Genetic factors People with particular human leukocyte antigen (HLA) types are at a higher risk of the condition. Immune factors HLAs are proteins that play a role in the body’s immune system. People with dermatomyositis show an abnormal immune reaction, whereby the body’s immune system attacks small blood vessels, causing inflammatory changes in the:musclesskinblood vessels Environmental factors Doctors observe this condition more frequently in females with exposure to high frequency UV radiation. Experts have also suggested that certain viruses could trigger the condition. These include:parvovirusenterovirusCoxsackie B virusechovirusHIV Medications Certain drugs can also trigger dermatomyositis. These include:anticancer drugsantibioticsnonsteroidal anti-inflammatory drugs Silicone breast implants or collagen insertions may also trigger or worsen the condition. Related conditions Dermatomyositis can increase a person’s risk of other conditions: Lung disease One-third of people with dermatomyositis have interstitial lung disease, which involves inflammation and scar tissue in the lungs. A person with respiratory muscle weakness may also develop aspiration pneumonia. Heart disease Weakness and inflammation of the heart muscle can lead to:myocarditiscongestive heart failurecoronary artery disease Connective tissue diseases Dermatomyositis can occur together with other connective tissue diseases, such as:rheumatoid arthritissystemic lupus erythematosusscleroderma Raynaud s disease This condition causes the sudden contraction of blood vessels in the hands and toes. The resulting interruption of blood flow can lead to:numbnesstinglingdiscoloration of digits Cancer Many people with dermatomyositis also have an underlying malignancy. Malignancies occur in 24% of cases of adult dermatomyositis. This risk is highest during the first 5 years following diagnosis. Diagnosis Doctors begin the diagnosis by looking at the person’s history, performing a thorough clinical examination, asking the person about their symptoms, and running different tests. Tests that they may request include:blood tests to check muscle enzymes such as creatine kinase and aldolaseblood tests to check for antibodies, including myositis-specific autoantibodieselectromyography to identify which muscle groups are most affected muscle and skin biopsyX-raysMRI scansbarium swallowcancer screening, which may involve:colonoscopyPap smearmammography Treatments The goal of treatment is to manage specific symptoms and address any other complications. Glucocorticoids: This is the first-line treatment for dermatomyositis. Glucocorticoids, particularly prednisolone, help reduce inflammation and swelling and suppress the body’s immune responses. Doctors initially prescribe a high dosage and then taper the drug slowly as the person’s muscle enzyme levels decline.Immunosuppressive medications: Doctors may also give other immunosuppressive drugs, such as methotrexate and azathioprine, to those with an insufficient response to steroids alone.Intravenous immunoglobulin (IVIG): Doctors may give people who are resistant to steroids IVIG, which they administer directly into the bloodstream. These blood products help boost the body’s immune system.Calcium channel blockers: These drugs remove calcium deposits and reduce the effects they may cause on the body.Calcification surgery: Surgical procedures can remove calcium deposits that are infected or painful.Physical therapy: This therapy improves muscle strength and prevents contractures.Speech therapy: This type of therapy helps people manage swallowing and speech difficulties. Nonsurgical treatments Other nonmedicinal management approaches may include:adding more protein to the dieta soft food diet for people with dysphagiaavoidance of or protection from the sunsleeping on an elevated surfacenot eating before bedtimegeneral rest Outlook and life expectancy There is no cure for dermatomyositis. The symptoms may come and go, and a person may require treatment for the rest of their life. Overall, about 10% of cases prove fatal, but the mortality rate is highest in the first year of the condition. The most common causes of death are:cancerischemic heart diseasepulmonary complications However, more than half of those who survive retain their usual level of strength, and 16% have no disability. With treatment, 1 in 5 people reach remission, while the rest continue with a polycyclic course. Dermatomyositis vs other conditions Dermatomyositis shares some similarities with other muscle and skin conditions, so doctors will need to consider the differences between these conditions when making a diagnosis. Dermatomyositis vs polymyositis Polymyositis is another inflammatory myopathy that causes muscle swelling, tenderness, and weakness. However, unlike dermatomyositis, polymyositis does not cause skin rashes. Dermatomyositis vs lupus Although a butterfly rash occurs in both conditions, rashes in the area below the nose — the nasolabial folds — are more common with dermatomyositis. Dermatomyositis affects the joint surface but not the interjoint spaces. However, people with cutaneous lupus have rashes in interjoint spaces. A scaly scalp is uncommon for people with cutaneous lupus, which does not usually involve the lower extremities. Dermatomyositis vs inclusion body myositis Inclusion body myositis produces symptoms similar to those of dermatomyositis, but it affects different muscle groups. For example, inclusion body myositis affects the wrists and fingers. Dermatomyositis vs scleroderma Scleroderma causes the excessive formation and accumulation of collagen. This condition results in skin thickening and hardening of the skin and specific organs. Scleroderma can occur with dermatomyositis. Summary Dermatomyositis is a rare muscular condition that requires lifelong treatment. This condition may lead to other health complications, affecting various body parts, such as the skin, lungs, and heart. A person should work with their doctor to create an effective treatment plan and prepare for possible complications. Last medically reviewed on September 20, 2022DermatologyPulmonary SystemBlood / HematologyGastroIntestinal / Gastroenterology 7 sourcescollapsedMedical News Today has strict sourcing guidelines and draws only from peer-reviewed studies, academic research institutions, and medical journals and associations. We avoid using tertiary references. We link primary sources — including studies, scientific references, and statistics — within each article and also list them in the resources section at the bottom of our articles. You can learn more about how we ensure our content is accurate and current by reading our editorial policy.Dermatomyositis. (2018). https://rarediseases.org/rare-diseases/dermatomyositis/Gara, S., et al. (2022). Juvenile dermatomyositis.https://www.ncbi.nlm.nih.gov/books/NBK534236/Jakubaszek, M., et al. (2015). Polymyositis and dermatomyositis as a risk of developing cancer.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4847280/Okogbaa, J., et al. (2019). Dermatomyositis: An acute flare and current treatments.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6582284/Qudsiya, Z., et al. (2022). Dermatomyositis. https://www.ncbi.nlm.nih.gov/books/NBK558917/Rad, S. N., et al. (2022). Malar rash.https://www.ncbi.nlm.nih.gov/books/NBK555981/Sporadic inclusion body myositis. (2019).https://rarediseases.org/rare-diseases/sporadic-inclusion-body-myositis/FEEDBACK:Medically reviewed by Megan Soliman, MD — By Rachel Ann Tee-Melegrito on September 20, 2022 Latest newsWhat sets 'SuperAgers' apart? Their unusually large neuronsOmega-3 may provide a brain boost for people in midlifeSeasonal affective disorder (SAD): How to beat it this fall and winterCDC: Monkeypox in the US 'unlikely to be eliminated in the near future'Why are more women prone to Alzheimer's? New clues arise Related CoverageEverything you need to know about myositisMedically reviewed by Nancy Carteron, M.D., FACR What is myositis? 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