What is Dermatomyositis?
Dermatomyositis is a rare autoimmune disease. It leads to an inflammatory response in which the body’s immune system attacks blood vessels in the muscle and skin. Typical symptoms include muscle weakness and a skin rash. Both children and adults are susceptible this disease, although it is most common in children between ages 5 and 10, and adults between ages 40 and 50. Highly experienced attorneys at MCT Law represent people with vaccine triggered dermatomyositis.
The adult form of the disease presents differently than the juvenile form. Additionally, females are affected about twice as often as males. However, 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 that about 3 out of one million children are diagnosed with juvenile dermatomyositis (known as JDM) each year.
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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. However, some individuals show a genetic predisposition to the condition, even though it’s not inherited in the way other muscle diseases are, such as muscular dystrophy.
It’s believed dermatomyositis is an abnormal immune system response triggered by some external factor, including infection, injury, sunburn or even some vaccinations. 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.
Attorneys at MCT Law are highly experienced at representing people with vaccine triggered dermatomyositis within 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 mainly shows itself in two ways.
First, a distinctive rash and muscle inflammation, and 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.
Second, muscle weakness caused by inflammation. The muscles affected are usually those closest to the ‘trunk’ of the body, which includes the neck, shoulders, and hips. So 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 detect elevated enzymes from inflamed muscles, as well as antinuclear antibodies. These results could be positive with dermatomyositis, as they are with other autoimmune conditions. An MRI detects subtle muscle inflammation and swelling which may help in the diagnosis of Dermatomyositis. 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 are often 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 magnifies the capillaries in the fingernail bed by using a special light. Distortion of blood vessels and abnormal swelling suggest active dermatomyositis.
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Although there is no cure for juvenile dermatomyositis, there are treatments to help reduce or eliminate 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.
- Corticosteroids, prednisone is the most commonly used; they work 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 it is used in a much smaller dose for dermatomyositis. Methotrexate may be given by pill or injection, but 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 sometimes causes 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 have their medications stopped within two years. About half of children diagnosed with JDM 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.