What is dermatomyositis?
Dermatomyositis (DM) refers to a group of muscular diseases also known as inflammatory myopathies. This specific and rare group of chronic diseases is uniquely characterized by chronic muscle and connective tissue inflammation paired with muscle weakness. In any autoimmune disease, antibodies attack healthy tissue, and in this case, healthy muscle, skin, and connective tissue. While this autoimmune disease is incurable, it is manageable through certain medical and holistic means.
DM causes degenerative changes in muscle and skin tissue, creating abnormalities and causing them to atrophy. Those with dermatomyositis may experience a wide range of symptoms that can affect their daily lives drastically. The body’s immune system is at war with itself and thus, management of DM will be a daily task for those suffering from the inflammatory muscle disease.
Signs and symptoms of dermatomyositis
The exhibition of symptoms is patient specific but certain occurrences happen more frequently. Symptoms can appear suddenly or gradually, and may come and go. Common symptoms of dermatomyositis include:
- Muscle weakness. The most common symptom of dermatomyositis is muscle weakness. This weakening typically affects the proximal muscles in the trunk of the body – hips, neck, shoulders, and the like. Over time, muscles can transition to feeling stiff, tender, or sore to completely losing mass, or atrophying. The loss of muscle strength, particularly in the upper arms or upper chest can also make daily tasks, including work, socialization, or the pursuit of physical activity extremely challenging.
- Skin rashes. Lesions and redness on the skin can occur before, after, or during muscle weakening and is a telltale sign of Dermatomyositis when paired with muscle atrophy. Rashes may present as increased pigmentation on the joints (Gottron’s sign) including areas around the knuckles, elbows, knees, and ankles. Rashes may also look like scaly skin and prevent the individual from sleeping or wearing clothes comfortably.
- Dysphagia or Dysphonia. The inability to swallow, difficulty swallowing, or the inability to speak can be a sign of DM. As the muscles in the throat, neck, and head become weakened, they can fail to execute these normal bodily processes.
- Changes in the skin. In addition to red, painful rashes, other epidermal abnormalities may occur. Hyperpigmentation, vitiligo, edema around the mucous membranes, and dilation of the capillaries in nail beds may be caused by DM. Blood vessels may also swell and become inflamed, causing a host of skin conditions.
- Calcium deposits. Children with juvenile dermatomyositis are especially prone to calcium deposits in the muscular tissue. These deposits may lead to muscular atrophy.
- Other symptoms may include malaise, low-grade fever, shortness of breath, weight loss, Raynaud phenomenon, altered gait, and hunched posture.
What are the complications and who is affected by dermatomyositis?
Depending on the severity of this rare disease, those experiencing the autoimmune disease may be met with a variety of complications. As these inflammatory myopathies affect the muscular system, any internal system and process associated with the body’s muscles can be drastically affected much to the detriment of the patient.
- Risk of falls. Our intricate muscular system helps us remain upright, walk downstairs, balance on one foot, and so much more. As muscles within the trunk weaken, simply walking can prove to be risky. As patients age, falling is more difficult to bounce back from and can lead to sprained, fractured, or broken bones, long hospital stays, and limited mobility.
- Lung problems. Muscle weakness can begin to progress outside of the trunk and proximal muscles, working its way towards the chest and back, affecting the lungs. Difficulty breathing, shortness of breath, and extreme fatigue can be a result of DM’s effects on the respiratory system.
- Stomach ulcers. As the disease progresses, stomach ulcers have been shown to form in 3-19% of patients with DM. Ulcers lead to further complications and can be evidence that the individual is no longer responding to immunosuppressive drugs. Some individuals that develop stomach ulcers succumb to secondary infections.
- Arrhythmia. The heart is itself a muscle that expands and contracts to pump blood throughout the body. As the muscle weakens, it can beat irregularly, which may eventually cause a heart attack.
- Increased risk of some cancers. Malignancy may occur before or in conjunction with Dermatomyositis. Studies show that cancer-associated DM is more likely to occur in patients over the age of 40 but there are no specific cancers with a proven association to DM. Scientists and medical doctors are still researching the association of malignancies with dermatomyositis
Who does DM affect?
It should be noted that Dermatomyositis is a rare disease, meaning that it affects less than 200,000 people a year in the United States. Adults and children are both susceptible to DM, however, in adults the inflammatory muscle disorder more often occurs in middle age (late 40’s to mid-60s). Women and African Americans are disproportionately affected by this muscle disease.
How does DM interact with other Conditions?
Dermatomyositis may cause other conditions to occur or may interact adversely with disorders presently in the body. Specifically, these conditions can be brought on or worsened for those with DM:
- Antisynthetase Syndrome (ASS). A large percentage of people diagnosed with ASS have either polymyositis or dermatomyositis. Antisynthetase syndrome may also initiate interstitial lung disease, Raynaud’s phenomenon, inflammatory polyarthritis, and mechanic’s hands.
- Other autoimmune disorders. Those suffering from an autoimmune disorder already – like Lupus, scleroderma, Sjorgden’s syndrome, or polymyositis can have exacerbated symptoms caused by DM and the other disease.
Appointments and Next Steps
As research continues, Dermatomyositis is becoming increasingly manageable with routine treatment. However, as the inflammatory muscle disease can affect a variety of bodily conditions, multiple medical departments may need to get involved. Rheumatologists, Dermatologists, and Endocrinologists can help diagnose and treat patients with this autoimmune disease.
Blood tests may prove effective in recognizing that an individual has an autoimmune disorder but may look similar to other illnesses or connective tissue disorders. Autoantibodies are telling of an autoimmune disease but not so specific that a doctor can conclusively link it to DM. Doctors may choose to diagnose the disorder through a number of methods, including a muscle biopsy.
Once diagnosed with DM, there are a number of ways to combat and suppress this autoimmune disorder.
- Immunosuppressive therapies. Administering high doses of corticosteroids, in particular prednisone, is a common treatment that quells symptoms associated with dermatomyositis. These medications work to reduce inflammation, swelling, and suppress the immune system’s responsive attack of antibodies. For those unresponsive to certain corticosteroid treatments, doses of azathioprine, methotrexate, tacrolimus, or cyclosporine may help suppress symptoms.
- Antimalarial medication. In conjunction with lower doses of glucocorticoids, antimalarial agents including hydroxychloroquine prove beneficial in treating rashes and other skin-related symptoms brought on by DM.
- Physical therapy. While this will not remedy inflammatory myopathies, it can help improve muscle strength and allow an individual to regain some autonomy over their own lives.
Because dermatomyositis can affect various parts of the body, the muscular system, and the skin, it’s important to consult with a team of doctors when seeking diagnosis and treatment. It’s crucial to establish a medical rapport and trusting relationship with a health care provider. UCF Health’s network of Orlando dermatologists are knowledgeable and experienced in diagnosing and treating a variety of skin conditions, including symptoms of dermatomyositis.
In order to manage any autoimmune disorder, medical help is necessary. Catching DM early on can help prevent other complications or disorders from developing and thus, drastically affecting one’s quality of life. Visit our patient portal today to learn more about autoimmune disorders, skin diseases, COVID-19 updates for patients, and more. You can even book an appointment through our online scheduling tool. We look forward to helping you get on a path to better health and wellness this year, and every year to follow.