DermatomyositisSymptoms, Doctors, Treatments, Advances & More
Learn About Dermatomyositis
Dermatomyositis is a disease that involves muscle inflammation and a skin rash. Polymyositis is a similar inflammatory condition that also involves muscle weakness, swelling, tenderness, and tissue damage but no skin rash. Both are part of a larger group of diseases called myopathies, more specifically inflammatory myopathies.
The cause of dermatomyositis is unknown. Experts think it may be due to a viral infection of the muscles or a problem with the body's immune system. It may also occur in people who have cancer in the abdomen, lung, or other parts of the body.
Anyone can develop this condition. It most often occurs in children age 5 to 15 and adults age 40 to 60. It affects women more often than men.
Symptoms may include:
- Muscle weakness, stiffness, or soreness
- Purple color to the upper eyelids
- Purple-red skin rash
- Shortness of breath
- Difficulty swallowing
The muscle weakness may come on suddenly or develop slowly over weeks or months. You may have trouble raising your arms over your head, getting up from a sitting position, and climbing stairs.
The rash may appear on your face, knuckles, neck, shoulders, upper chest, and back.
The initial treatment is the use of corticosteroid medicines. The dose of medicine is slowly tapered off as muscle strength improves. This takes about 4 to 6 weeks. You may stay on a low dose of a corticosteroid medicine after that.
Medicines to suppress the immune system may be used to replace the corticosteroids. These medicines may include azathioprine, methotrexate or mycophenolate.
Treatments that may be tried when disease that remains active in spite of these medicines are:
- Intravenous gamma globulin
- Biologic medicines
When your muscles get stronger, your provider may tell you to slowly cut back on your doses. Many people with this condition must take a medicine called prednisone for the rest of their lives.
If a tumor is causing the condition, the muscle weakness and rash may get better when the tumor is removed.
UPMC Arthritis And Autoimmunity Center
Chester V. Oddis, MD is a noted researcher in the clinical, epidemiological and serologic description of the idiopathic inflammatory myopathies. He has completed epidemiological studies on the incidence of polymyositis and dermatomyositis as well as longitudinal studies on functional disability in myositis patients. His focus has been in the autoantibody patterns in patients with inflammatory myopathy and their peculiar immunogenetic associations.Dr. Oddis has written extensively on the diagnosis and management of patients with myositis. He has published and lectured on the use of novel immunosuppressive agents such as tacrolimus (formerly FK506) in the treatment of patients with refractory myositis. This represents the only reported experience of this particular drug in the pharmacologic treatment of inflammatory myopathy. In collaboration with the Department of Neurology, he has assessed the efficacy and utility of outpatient needle muscle biopsy in the diagnosis of inflammatory myopathy. This is important given the cost-containment focus of the current health-care market.Dr. Oddis is the co-investigator on an important national NIH funded study on the efficacy of doxycycline in preventing the progression of knee osteoarthritis in middle-aged women. This study is the first of its kind in assessing the effect of a disease-modifying agent in osteoarthritis.Board certified in internal medicine and rheumatology, Dr. Oddis received his medical degree from Pennsylvania State University, Milton S. Hershey Medical Center and his undergraduate degree from the University of Pittsburgh. He completed his residency in internal medicine at Penn State and fellowship at the University of Pittsburgh Medical Center. Dr. Oddis is rated as an Elite provider by MediFind in the treatment of Dermatomyositis. His top areas of expertise are Dermatomyositis, Myositis, Polymyositis, and Antisynthetase Syndrome.
Johns Hopkins Bayview Medical Center
Dr. Julie Paik completed her medical school training at the George Washington University School of Medicine in Washington, DC. She then completed her residency and chief residency at UCLA-Cedars-Sinai/VA Medical Center in Los Angeles, California. She completed rheumatology fellowship at Johns Hopkins and joined the faculty in 2013. She currently sees patients at the Johns Hopkins Myositis Center and Scleroderma Center. Her research interests are in myositis and scleroderma or overlap myositis. She also has a special interest in novel therapeutics in refractory dermatomyositis. This provider is registered with the Florida Department of Health to perform telehealth services for patients in Florida. Dr. Paik is rated as an Elite provider by MediFind in the treatment of Dermatomyositis. Her top areas of expertise are Dermatomyositis, Polymyositis, Myositis, and Scleroderma.
Johns Hopkins Bayview Medical Center
Andrew Mammen is a Neuromusculoskeletal Medicine specialist and a Neurologist in Baltimore, Maryland. Dr. Mammen is rated as an Elite provider by MediFind in the treatment of Dermatomyositis. His top areas of expertise are Myositis, Dermatomyositis, Polymyositis, and Necrotizing Myopathy (NM).
It is important to be followed by a health care provider when you have dermatomyositis. Severe illness can lead to disability and can sometimes result in death.
Symptoms may go away completely in some people, such as children.
The condition may be fatal in adults due to:
- Severe muscle weakness
- Malnutrition
- Pneumonia
- Lung failure
The major causes of death with this condition are cancer and lung disease.
People with lung disease with the anti-MDA-5 antibody have a poor prognosis in spite of current treatment.
Complications may include:
- Lung disease
- Acute renal failure
- Cancer (malignancy)
- Inflammation of the heart
- Joint pain
Contact your provider if you have muscle weakness or other symptoms of this condition.
Summary: This phase Ib trial studies the side effects of nivolumab and to see how well it works in treating patients with autoimmune disorders and cancer that has spread to other places in the body or cannot removed by surgery. Immunotherapy with monoclonal antibodies, such as nivolumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and sprea...
Summary: The purpose of this long-term follow-up (LTFU) study is to collect delayed adverse events (AEs) and understand the persistence of KYV-101 (autologous CAR T cell product; gene-modified product), in participants who have been administered KYV-101 (gene-modified product; autologous CAR T cell product). This LTFU protocol will be open to any participant who received at least one infusion of KYV-101 in...
Published Date: January 28, 2025
Published By: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Dinulos JGH. Connective tissue diseases. In: Dinulos JGH, ed. Habif's Clinical Dermatology. 7th ed. Philadelphia, PA: Elsevier; 2021:chap 17.
Greenberg SA. Inflammatory myopathies. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 248.
Nagaraju K, Aggarwal R, Lundberg IE. Inflammatory diseases of muscle and other myopathies. In: Firestein GS, McInnes IB, Koretzky GA, Mikuls TR, Neogi T, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 12th ed. Philadelphia, PA: Elsevier; 2025:chap 86.
National Organization for Rare Disorders website. Dermatomyositis. rarediseases.org/rare-diseases/dermatomyositis/. Updated May 4, 2018. Accessed February 27, 2025.