Dermatomyositis

Title

Other Names:

Adult dermatomyositis

Categories:

Kidney and Urinary Diseases; Nervous System Diseases; Skin Diseases

Summary Summary

Dermatomyositis is one of a group of acquired muscle diseases called inflammatory myopathies (disorder of muscle tissue or muscles), which are characterized by chronic muscle inflammation accompanied by muscle weakness. The cardinal symptom is a skin rash that precedes or accompanies progressive muscle weakness.^[1] Dermatomyositis may occur at any age, but is most common in adults in their late 40s to early 60s, or children between 5 and 15 years of age.^[2] There is no cure for dermatomyositis, but the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest.^[1] The cause of dermatomyositis is unknown.^[3]

Last updated: 8/26/2013

Symptoms Symptoms

The signs and symptoms of dermatomyositis may appear suddenly or develop gradually, over weeks or months.^[2]^[3] The cardinal symptom of dermatomyositis is a skin rash that precedes or accompanies progressive muscle weakness.^[1] The rash looks patchy, with bluish-purple or red discolorations, and characteristically develops on the eyelids and on muscles used to extend or straighten joints, including knuckles, elbows, heels, and toes. Red rashes may also occur on the face, neck, shoulders, upper chest, back, and other locations, and there may be swelling in the affected areas.^[1]^[3] The rash sometimes occurs without obvious muscle involvement.^[1]

Adults with dermatomyositis may experience weight loss or a low-grade fever, have inflamed lungs, and be sensitive to light. Children and adults with dermatomyositis may develop calcium deposits, which appear as hard bumps under the skin or in the muscle (called calcinosis). Calcinosis most often occurs 1-3 years after the disease begins. These deposits are seen more often in children with dermatomyositis than in adults. In some cases of dermatomyositis, distal muscles (muscles located away from the trunk of the body, such as those in the forearms and around the ankles and wrists) may be affected as the disease progresses. Dermatomyositis may be associated with collagen-vascular or autoimmune diseases, such as lupus. ^[1]

Last updated: 8/26/2013

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Abnormal eyelid morphology	Abnormality of the eyelid Abnormality of the eyelids [ more ]	0000492
Autoimmunity	Autoimmune disease Autoimmune disorder [ more ]	0002960
EMG abnormality		0003457
Erythema		0010783
Inflammatory myopathy		0009071
Myalgia	Muscle ache Muscle pain [ more ]	0003326
Periorbital edema	Puffiness around the eyes Puffy eyes Swelling around the eyes [ more ]	0100539
Proximal muscle weakness		0003701
30%-79% of people have these symptoms
Abnormal hair quantity		0011362
Abnormality of the nail		0001597
Acrocyanosis		0001063
Arthralgia	Joint pain	0002829
Arthritis	Joint inflammation	0001369
Chondrocalcinosis		0000934
Diffuse reticular or finely nodular infiltrations		0002207
Dry skin		0000958
Fatigue	Tired Tiredness [ more ]	0012378
Interstitial pulmonary abnormality		0006530
Muscular hypotonia	Low or weak muscle tone	0001252
Papule		0200034
Pruritus	Itching Itchy skin Skin itching [ more ]	0000989
Pulmonary fibrosis		0002206
Recurrent respiratory infections	Frequent respiratory infections Multiple respiratory infections respiratory infections, recurrent Susceptibility to respiratory infections [ more ]	0002205
Respiratory insufficiency	Respiratory impairment	0002093
Skin ulcer		0200042
Weight loss		0001824
5%-29% of people have these symptoms
Abnormality of eosinophils		0001879
Aplasia/Hypoplasia of the skin	Absent/small skin Absent/underdeveloped skin [ more ]	0008065
Breast carcinoma	Breast cancer	0003002
Cellulitis	Bacterial infection of skin Skin infection [ more ]	0100658
Cutaneous photosensitivity	Photosensitive skin Photosensitive skin rashes Photosensitivity Sensitivity to sunlight Skin photosensitivity Sun sensitivity [ more ]	0000992
Dysphonia	Inability to produce voice sounds	0001618
Feeding difficulties in infancy		0008872
Fever		0001945
Gangrene		0100758
Gastrointestinal stroma tumor		0100723
Lung adenocarcinoma		0030078
Lymphoma		0002665
Myocardial infarction	Heart attack	0001658
Myocarditis		0012819
Pericarditis		0001701
Pulmonary arterial hypertension		0002092
Sinus tachycardia		0011703
Telangiectasia of the skin		0100585
Vasculitis		0002633

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Last updated: 6/1/2018

Cause Cause

The cause of this disorder is unknown. It is theorized that an autoimmune reaction (reactions caused by an immune response against the body's own tissues) or a viral infection of the skeletal muscle may cause the disease.^[3] In addition, some doctors think certain people may have a genetic susceptibility to the disease.^[2]

Last updated: 8/26/2013

Treatment Treatment

While there is no cure for dermatomyositis, the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. The standard treatment for dermatomyositis is a corticosteroid drug, given either in pill form or intravenously. Immunosuppressant drugs, such as azathioprine and methotrexate, may reduce inflammation in people who do not respond well to prednisone. Periodic treatment using intravenous immunoglobulin can also improve recovery. Other immunosuppressive agents used to treat the inflammation associated with dermatomyositis include cyclosporine A, cyclophosphamide, and tacrolimus. Physical therapy is usually recommended to prevent muscle atrophy and to regain muscle strength and range of motion. Many individuals with dermatomyositis may need a topical ointment, such as topical corticosteroids, for their skin disorder. They should wear a high-protection sunscreen and protective clothing. Surgery may be required to remove calcium deposits that cause nerve pain and recurrent infections.^[1]

Last updated: 8/26/2013

Prognosis Prognosis

Most cases of dermatomyositis respond to therapy. Some people may recover and have symptoms completely disappear. This is more common in children. In adults, death may result from severe and prolonged muscle weakness, malnutrition, pneumonia, or lung failure. The outcome is usually worse if the heart or lungs are involved. ^[1]^[3]

Last updated: 8/26/2013

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Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Dermatomyositis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
The Research Portfolio Online Reporting Tool (RePORT) provides access to reports, data, and analyses of research activities at the National Institutes of Health (NIH), including information on NIH expenditures and the results of NIH-supported research. Although these projects may not conduct studies on humans, you may want to contact the investigators to learn more. To search for studies, enter the disease name in the "Text Search" box. Then click "Submit Query".

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Myositis Support and Understanding
9125 N. Old State Road
Lincoln, DE 19960
Telephone: 302-339-3241
E-mail: info@UnderstandingMyositis.org
Website: http://www.understandingmyositis.org/
Myositis UK
146 Newtown Road
Woolston
Southampton
SO19 9HR
United Kingdom
Telephone: 023 8044 9708
E-mail: msg@myositis.org.uk
Website: http://www.myositis.org.uk/
The Myositis Association
1940 Duke Street
Suite 200
Alexandria, VA 22314
Toll-free: 800-821-7356
Telephone: 703-299-4850
Fax: 703-535-6752
E-mail: TMA@myositis.org
Website: http://www.myositis.org

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

The Assistance Fund provides various services, including education and financial aid, to help patients with a chronic or serious illness cover the cost of FDA-approved medications. Patients must be U.S citizens or permanent residents.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

MayoClinic.com provides information about dermatomyositis. Click on the link above to access this information.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Dermatomyositis. Click on the link to view a sample search on this topic.

Selected Full-Text Journal Articles

Koler RA, Montemarano A. Dermatomyositis. American Family Physician. 2001; 64: 1565-72. Available at: http://www.aafp.org/afp/20011101/1565.html. Accessed November 4, 2009.

Related Diseases Related Diseases

The following diseases are related to Dermatomyositis. If you have a question about any of these diseases, you can contact GARD.

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My father was diagnosed with dermatomyositis and has some long-lasting symptoms. Is it possible that radiation exposure could be contributing to his condition? See answer
My mother has been diagnosed with dermatomyositis. She has not responded to the treatments she has received. Can you provide information about the symptoms, treatment, and prognosis for this condition? See answer

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