Zack has had a rash on his face for about a month and with no resolve from his doctors best efforts she sent us to Stanford for more test. First we saw a Dermatologist and they diagnosed him with a rare muscle disease called Dermatomyositis. Which is said Der-my-toe-my-o-s-eye-tis. They referred us to Rheumatology. We saw them on Friday and while we were there they did some physical test and lots of blood work. That evening they called with the results to the blood test and confirmed that he did have this disease. They said that the results could have been a lot worse than what they are so they hope they caught it early. So what does this all mean? Well, he now has to be on steroids for at least 2 years. They are starting him on a high dose at first and than it will taper off as times go by. After 2 years they will retest him and if all is well he will be in remission. This disease is not curable but maintainable. So we are expecting some side affects from the steroids he is taking but were staying positive with him. He will also be in physical therapy during this time. We have to go to Stanford every other week for a physical and weekly for therapy.
So what is Dermatomyositis? I have copy and pasted some info below from a medical web-site. Oh and that's another thing. The Dr. has told us to stay away from the internet on this so we don't get discouraged hearing/reading horror stories. So if you do any looking up on this we love to hear any questions you have or any we should ask the Dr. but we don't want to hear any horror stories. Sorry some of you in my Family are finding out this way but we are trying to soak this all in too.
Definition of Dermatomyositis
Dermatomyositis: A chronic inflammatory disease of skin and muscle which is associated with patches of slightly raised reddish or scaly rash. The rash can be on the bridge of the nose, around the eyes, or on sun-exposed areas of the neck and chest. Classically, however, it is over the knuckles. When the characteristic inflammation of the muscle (myositis) occurs without skin disease, the condition is referred to as polymyositis.
Dermatomyositis is one of a group of acquired muscle diseases called inflammatory myopathies. The disease has a subacute (somewhat short and relatively severe) onset. It affects both children and adults. Females are more often affected than males. Dermatomyositis is characterized by a rash accompanying, or more often, preceding muscle weakness. The most common symptom is muscle weakness, usually affecting those muscles that are closest to the trunk of the body (proximal). Eventually, patients have difficulty rising from a sitting position, climbing stairs, lifting objects, or reaching overhead. In some cases, distal muscles (those not close to the trunk of the body) may be affected later in the course of the disease. Trouble with swallowing (dysphagia) may occur. Occasionally, the muscles ache and are tender to touch. Some patients develop hardened bumps of calcium deposits under the skin. Patients may also feel fatigue and discomfort and have weight loss or a low-grade fever.
Treatment commonly involves a steroid drug called prednisone. For patients in whom prednisone is not effective, other immunosuppressants such as azathioprine and methotrexate may be prescribed. Recently, a drug called intravenous immunoglobulin was shown to be effective and safe in the treatment of the disease. Physical therapy is usually recommended to preserve muscle function and avoid muscle atrophy. Most cases of dermatomyositis respond to therapy. The disease is usually more severe and resistant to therapy in patients with cardiac or pulmonary problems.
Both polymyositis and dermatomyositis can sometimes be associated with cancers, including lymphoma, breast, lung, ovarian, and colon cancer. The cancer risk is reported to be much greater with dermatomyositis than polymyositis. (See polymyositis).
What is polymyositis?Polymyositis is a disease of muscle featuring inflammation of the muscle fibers. The cause of the disease is not known. It begins when white blood cells, the immune cells of inflammation, spontaneously invade muscles. The muscles affected are typically those closest to the trunk or torso. This results in weakness that can be severe. Polymyositis is a chronic illness with periods of increased symptoms, called flares or relapses, and minimal or no symptoms, known as remissions.
Polymyositis is slightly more common in females. It affects all age groups, although its onset is most common in middle childhood and in the twenties. Polymyositis occurs throughout the world. Polymyositis can be associated with skin rash and is then referred to as "dermatomyositis." It also can affect other areas of the body and is, therefore,
referred to as a systemic illness. Occasionally, it is associated with cancer, or with other diseases of connective tissue (see systemic lupus erythematosus, scleroderma and rheumatoid arthritis).
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