Dermatomyositis

Title

Other Names:

Adult dermatomyositis

Categories:

Kidney and Urinary Diseases; Nervous System Diseases; Skin Diseases

Subtypes:

Juvenile dermatomyositis; Juvenile polymyositis

This disease is grouped under:

Idiopathic inflammatory myopathy

Summary Summary

Dermatomyositis is an autoimmune condition that causes skin changes and muscle weakness. Symptoms can include a red skin rash around the eyelids, red bumps around the joints, and muscle weakness in the arms and legs. Dermatomyositis is most common in adults between ages 40 and 60, or in children between ages 5 and 15. Muscle weakness gets worse over time and can lead to stiff joints and muscle wasting. The cause for dermatomyositis is unknown. Diagnosis is made through a clinical exam and microscopic examination of a piece of skin and muscle. Treatment is focused on managing the symptoms. Options include different medications, physical therapy, and exercise.^[1]^[2]^[3]^[4]

Last updated: 6/11/2020

Symptoms Symptoms

The following list includes the most common signs and symptoms in people with dermatomyositis. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.

Signs and symptoms may include:^[3]^[4]

A reddish-purple rash around the eyelids
Red or violet bumps that form on the outside joints of the hand (Gottron papules)
Red or violet bumps on the knees and elbows
Discolored skin on shoulders, neck, upper back (shawl sign)
Muscle weakness starting in the arms and/or legs
Joint pain

The symptoms of dermatomyositis may appear suddenly or develop gradually. The first symptom of dermatomyositis is a skin rash that may come before or with the muscle weakness. Other organs may be affected such as the lungs and digestive tract. The muscle weakness gets worse over time and can lead to rigid joints, pain, and muscle wasting.^[4]

Last updated: 6/11/2020

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.

Showing of 44 |

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		0100539
Proximal muscle weakness	Weakness in muscles of upper arms and upper legs	0003701
30%-79% of people have these symptoms
Abnormal hair quantity		0011362
Abnormality of the nail		0001597
Acrocyanosis	Persistent blue color of hands, feet, or parts of face	0001063
Arthralgia	Joint pain	0002829
Arthritis	Joint inflammation	0001369
Chondrocalcinosis	Calcium deposits in joints	0000934
Diffuse reticular or finely nodular infiltrations		0002207
Dry skin		0000958
Fatigue	Tired Tiredness [ more ]	0012378
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	Open skin sore	0200042
Weight loss		0001824
5%-29% of people have these symptoms
Abnormal eosinophil morphology		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	Death of body tissue due to lack of blood flow or infection	0100758
Gastrointestinal stroma tumor		0100723
Lung adenocarcinoma		0030078
Lymphoma	Cancer of lymphatic system	0002665
Myocardial infarction	Heart attack	0001658
Myocarditis	Inflammation of heart muscle	0012819
Pericarditis	Swelling or irritation of membrane around heart	0001701
Pulmonary arterial hypertension	Increased blood pressure in blood vessels of lungs	0002092
Sinus tachycardia		0011703
Telangiectasia of the skin		0100585
Vasculitis	Inflammation of blood vessel	0002633

Showing of 44 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 2/1/2021

Cause Cause

The cause of this disorder is unknown. It is thought to be due to a combination of genetic and environmental factors.^[3]

Last updated: 6/11/2020

Diagnosis Diagnosis

Dermatomyositis is diagnosed through a clinical exam for specific signs and symptoms. A standard list of symptoms is available for dermatomyositis.^[5] A skin and muscle biopsy may be done to get a small piece of tissue to examine under the microscope.^[2]^[3]

Last updated: 6/11/2020

Treatment Treatment

Treatment for dermatomyositis is focused on managing the symptoms. The standard treatment for dermatomyositis is a corticosteroid drug. Other options include immunosuppressant medications, physical therapy, and exercise. Many individuals with dermatomyositis may need a topical ointment for the skin rash.^[1]

Specialists involved in the care of someone with dermatomyositis may include:

Dermatologist
Neurologist
Physical therapist
Orthopedist

Last updated: 6/11/2020

Statistics Statistics

It has been estimated that about 10 in 1,000,000 people are diagnosed every year with dermatomyositis. The exact number of people with dermatomyositis is unknown.^[1]^[6]

Last updated: 6/11/2020

Do you have updated information on this disease? We want to hear from you.

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.

Patient Registry

The Autoimmune Registry supports research for Dermatomyositis by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

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

Cure JM Foundation
P.O. Box 45768
Baltimore, MD 21297
Telephone: (760) 487-1079
E-mail: info@curejm.org
Website: http://www.curejm.org/
Myositis Support and Understanding
9125 N. Old State Road
Lincoln, DE 19960
Toll-free: 1-888-MYO-RARE (696-7273)
Telephone: +1-302-339-3241
E-mail: info@UnderstandingMyositis.org
Website: https://understandingmyositis.org/
Myositis UK
146 Newtown Road
Woolston
Southampton
SO19 9HR
United Kingdom
Telephone: 023 8044 9708
E-mail: msg@myositis.org.uk
Website: https://www.myositis.org.uk/
The Myositis Association (TMA)
1940 Duke Street
Suite 200
Alexandria, VA 22314
Toll-free: 1-800-821-7356
Telephone: +1-703-299-4850
Fax: +1-703-535-6752
E-mail: TMA@myositis.org
Website: https://www.myositis.org/

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
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.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References

Sasaki H, Kohsaka H. Current diagnosis and treatment of polymyositis and dermatomyositis. Mod Rheumatol. 2018; 28(6):913-921. https://pubmed.ncbi.nlm.nih.gov/29669460.
Chu LL, Rohekar G. Dermatomyositis. CMAJ. 2019; 191(12):E340. https://pubmed.ncbi.nlm.nih.gov/30910882.
Bogdanov I, Kazandjieva J, Darlenski R, Tsankov N. Dermatomyositis: Current concepts. Clin Dermatol. 2018; 36(4):450-458. https://pubmed.ncbi.nlm.nih.gov/30047429.
Sena P, Gianatti A, Gambini D. Dermatomyositis: clinicopathological correlations. G Ital Dermatol Venereol. 2018; 153(2):256-264. https://pubmed.ncbi.nlm.nih.gov/29368856.
Lundberg IE, Tjärnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups [published correction appears in Ann Rheum Dis. 2018 Sep;77(9):e64]. Ann Rheum Dis. 2017; 76(12):1955-1964. https://pubmed.ncbi.nlm.nih.gov/29079590.
Strowd LC, Jorizzo JL. Review of dermatomyositis: establishing the diagnosis and treatment algorithm. Jl of Derm Treatment. 2013; 24(6):418-421. https://pubmed.ncbi.nlm.nih.gov/22686682.