WHAT IS DERMATOMYOSITIS?
Dermatomyositis is a rare autoimmune disease that causes an inflammatory response in which the body’s immune system attacks blood vessels in the muscle and skin. The characteristic symptoms are a skin rash and muscle weakness. Both children and adults can get this disease; it’s most common for children ages 5 -10 and adults ages 40-50. Maglio Christopher & Toale, P. A. is experienced at representing people with vaccine triggered dermatomyositis in the United States Court of Federal Claims.
The course and presentation of the disease are different in adults than in children with the juvenile form of the disease. Females are affected about twice as much as males. It does not appear that people of a certain race or from a specific geographic region are predisposed to this disease. The American College of Rheumatology estimates about three in one million children are diagnosed with juvenile dermatomyositis (known as JDM) each year.
What Causes Dermatomyositis (JDM) and Could Vaccines be a Factor?
Doctors and researchers don’t fully understand what causes this disease; no single factor has been identified as the cause of dermatomyositis or JDM. Some individuals may have a genetic predisposition to the condition, but it’s not inherited in the way of other muscle diseases, such as muscular dystrophy.
It’s believed dermatomyositis is an abnormal immune system response triggered by some external factor, including infection, injury, sunburn or possibly immunizations. According to Boston Children’s Hospital, a vaccine may trigger a child’s JDM if he or she was predisposed to the condition. No specific vaccine has been identified as a singular trigger, but reactions have been reported in medical literature after the H1N1 vaccine, trivalent influenza vaccine, and HBV or Hepatitis B vaccination.
Maglio Christopher & Toale, P. A. is experienced at representing people with vaccine triggered dermatomyositis in the United States Court of Federal Claims. You can download and read through one of our more recent case results here: Case 00-749V.
What are the Symptoms of Dermatomyositis?
The disease manifests itself in two main ways: A distinctive rash and muscle inflammation. A pinkish/purple or reddish rash usually appears on the face, eyelids, and hands, and sometimes the skin near joints, including the knuckles, knees, and elbows. The rash is often scaly and may look similar to eczema, but the color should set it apart from other skin conditions. This rash may get worse after sun exposure and this is often the first sign of dermatomyositis.
Muscle weakness caused by inflammation is the other hallmark symptom of dermatomyositis. The muscles affected are usually those closest to the trunk, including around the neck, shoulders, and hips. Patients may have a hard time getting into a car, off the floor, climbing stairs or brushing their hair.
- Difficulty swallowing
- Muscle pain or tenderness
- Fatigue, fever and weight loss
- Hardened deposits of calcium under the skin
- Stomach ulcers and intestinal tears
- Lung problems
Blood tests can detect elevated enzymes from inflamed muscles, as well as antinuclear antibodies. These results may be positive with this disease, as with other autoimmune conditions. Magnetic Resonance Imaging, or MRI, can detect subtle muscle inflammation and swelling. MRI’s allow doctors to look at a full muscle and identify patterns or patches of inflammation.
- Muscle biopsies are used to remove and study a small piece of the muscle. In dermatomyositis, the capillary blood vessels in the muscle often are damaged and surrounded by inflammatory cells.
- Electromyography is a specialized procedure that allows a doctor to insert a small needle into a muscle; electrical activity is measured as the patient flexes and relaxes the muscle. Changes in the pattern of electrical activity can indicate dermatomyositis or JDM.
- Nailfold capillaroscopy can be used to magnify the capillaries in the fingernail bed by using a special light. Distortion of blood vessels and abnormal swelling suggest active dermatomyositis.
Although there is no cure for juvenile dermatomyositis, there are treatments that can help reduce or eliminate the symptoms. There are three possible outcomes for those diagnosed with JDM: continuous disease, chronic limitations or full remission. The most common medications used are corticosteroids, immunosuppressants, and chemotherapy.
- Prednisone is the most commonly used corticosteroid; it works quickly to suppress the immune system and helps control muscle, joint, and skin inflammation. As the condition improves, the dose will be lowered to help reduce the side effects of steroids, which include weight gain, increased risk of infection and facial swelling.
- Methotrexate may be used in patients whose symptoms are not controlled by prednisone or as a supplemental treatment to corticosteroids. This drug is used to treat cancer, but in dermatomyositis, it is used in a much smaller dose. Methotrexate may be given by pill or injection and can cause mouth sores and upset stomach.
- Hydroxychloroquine is used to treat the rash associated with dermatomyositis. In a small percentage of patients, this drug can cause pigment to accumulate in the retina of the eye, so those who take it need to be monitored by an ophthalmologist.
- Intravenous immunoglobulin consists of purified antibodies, which are proteins used by the immune system to fight infection. IVIG is administered intravenously and has been shown to control the inflammatory process.
When the above therapies don’t work, other treatment options include immune suppressive drugs cyclosporine and cyclopentolate mofetil.
What is the prognosis for those with JDM?
Most children go into remission and may have their medications eliminated within two years. About half of children diagnosed with JDM will recover completely. Some children may have a form of the disease that does not respond well to medications; others may have repeat episodes of the disease. Secondary conditions that have been seen with JDM include arthritis, diabetes, and celiac disease. Overall, most children diagnosed with JDM go on to live active lives.