Dermatomyositis is an inflammatory condition characterized by skin rash and muscular weakness. It has been observed in children aged 5 to 15 years, as well as people aged late 40s to early 60s. The symptoms appear gradually over a few weeks to a few months.
The precise cause of dermatomyositis is unknown, and the elements that may contribute to it are still being researched. The possible causes include genetic factors, autoimmune disorders, cancer, toxic and infectious agents, and certain medicines such as penicillamine, statins, hydroxyurea, phenylbutazone, and quinidine.
Symptoms
A spotty rash with bluish purple discolorations is the initial symptom. The eyelids, face, knees, knuckles, elbows, back, and chest develop a red or violet rash. Weakness develops in the muscles of the legs, hips, neck, upper arms, and shoulders, which worsens over time. Fever, weakness, weight loss, calcinosis in children (hard calcium deposits in the skin), intestinal perforations, gastrointestinal ulcers in children, and lung issues are among the other symptoms.
Complications
One of the problems is the weakness of the esophageal muscles, which causes trouble swallowing food (dysphagia) and can lead to malnutrition and weight loss. Because of dysphagia, liquids, meals, and saliva may enter the lungs and cause pneumonia. Ulcers in the stomach and intestines can cause them to bleed. Infections of the gut and respiratory systems are more likely to occur. Calcinosis is the formation of calcium deposits in connective tissue, skin, and muscles.
Dermatomyositis-related medical disorders include:
- Myocarditis (inflammation of heart muscle)
- Raynaud’s phenomenon (cold temperatures produce paleness in the fingertips, cheeks, nose, toes, and ears)
- Sjogren’s syndrome (autoimmune disease affecting the salivary and tear glands)
- Scleroderma is an autoimmune condition that causes skin thickening
- Rheumatoid arthritis (an inflammatory illness that causes joint swelling)
- Lupus (an autoimmune illness that affects all organs)
- Interstitial lung disease (affects the space around the lung’s air sacs and tissue)
- Cancers of the gastrointestinal system, the ovaries, the breasts, the pancreas, the lungs, and the cervix
- It causes stillbirth or early birth during pregnancy
Diagnosis
The key diagnostic symptom of dermatomyositis is the distinctive rash. The cross-sectional images of the muscles are generated using an MRI scan (magnetic resonance imaging), which generates images using a magnetic field and radio waves. MRI can identify calcification, fibrosis, muscular edema, and patterns of muscle weakening. The doctor may do an electromyography, which measures electrical activity as the patient contracts and relaxes the muscle. If there are alterations in the pattern of electrical activity, the test is positive for a muscle condition. The distribution of the disease can also be identified by electromyography, which tests the different muscles.
A small sample of muscle is taken (biopsy) and tested to detect injury, infection, muscle edema, enzyme shortages, and aberrant proteins. A blood test is performed to determine the levels of muscle enzymes such as aldolase and creatinine, as well as to discover antibodies specific to dermatomyositis. An increase in muscle enzymes implies muscular injury. Dermatomyositis can also be diagnosed with a skin biopsy.
Treatment
The treatment is intended to improve muscle strength and function. Corticosteroids, such as prednisone, are used to reduce muscle swelling and the formation of antibodies. Although improvement can be visible in 2 to 4 weeks, the medicine must be taken for years. Corticosteroids used topically to the skin are also advised. To supress the negative effects of corticosteroids (disturbance in sleep, weight gain, increased hunger, and psychological issues), the doctor prescribes bisphosphonates such as risedronate, zoledronic acid, alendronate, and supplements such as vitamin D and calcium. Immunosuppressive medications such as cyclosporine (sandimmune, neoral) and cyclophosphamide (cytoxan) are used to treat the symptoms of interstitial lung disease and dermatomyositis. Chloroquine (aralen) and hydroxychloroquine (plaquenil) are antimalarial medications. Over-the-counter drugs such as acetaminophen (Tylenol), ibuprofen (motrin, advil), and aspirin can be used to treat pain. Antibodies are extracted from blood donors and administered intravenously to combat the antibodies that attack the skin and muscle in dermatomyositis. Tacrolimus (Prograf) is a transplant rejection medication. Rituximab (Rituxan) is administered to improve muscle strength and minimize symptoms such as skin rashes. When calcium deposits become excruciatingly uncomfortable, surgery to remove them is advised.
Speech therapy is offered to patients who have developed jaw muscle weakness and are having difficulties swallowing. A physical therapist offers the patient exercises that can assist in enhancing his/her muscle condition in order to increase muscle strength and flexibility. Since chewing and swallowing become difficult in dermatomyositis, the dietician demonstrates how to prepare easily chewable foods for the patient.
Prevention
There is no precise way to avoid dermatomyositis, but regular exercise can help build strong and flexible muscles. After consultation with the physical therapist, an exercise plan must be developed. Sunscreen lotion and protective clothes should be worn to protect the skin from the sun.
