Juvenile Dermatomyositis

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Juvenile Dermatomyositis (JDMS) is a disease that causes a skin rash and weak muscles in children. The skin rash and weak muscles of JDMS are caused by inflammation in the blood vessels that lie under the skin and in the muscles. This is called vasculitis. Since blood vessels run throughout the body, JDMS can also affect other areas such as the digestive tract. Usually, the worst symptoms are in the skin and muscles.

JDMS is often called an autoimmune disease. The immune system is a group of cells which normally protects the body from infections. In autoimmune diseases, these cells are signaled or triggered to "turn on" their infection-fighting process, but then can't turn it off. The process then ends up damaging the body rather than protecting it.

One way the immune system cells fight infection is by a process called inflammation. But when the cells can't "turn off" the inflammation process, tissues are damaged. In JDMS, blood vessels in the skin and muscles are inflamed. An inflamed muscle will feel weak or sore, while inflamed skin will have a rash.

The symptoms of juvenile dermatomyositis often appear gradually. At times, there may be a more acute, or intense, onset of symptoms. The following are the most common symptoms of JDMS. However, each child may experience symptoms differently. Symptoms may include:

  • fever
  • rash around the eyelids and/or knuckles and finger joints; a rash may also occur on the elbows, knees, and ankles
  • muscle weakness
  • fatigue
  • malaise
  • muscle pain and tenderness
  • irritability
  • weight loss (due to difficulty swallowing)
  • joint pain and inflammation
  • calcium deposits under the skin
  • mouth ulcers

  • Symptoms of juvenile dermatomyositis may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.

    JDMS is very rare, and its cause remains unknown, however, there is scientific thought that the condition may arise in children who have a genetic predisposition causing problems clearing certain viruses from the body.

    . . . Phases of JDMS
    The course of juvenile dermatomyositis is often divided into four phases, based on symptoms and findings on examination. The four phases are briefly described below:

  • Prodromal period This phase can last for weeks or months. The symptoms seen during this period are nonspecific
  • Progressive muscle weakness and rash In this phase, muscle weakness increases for days and weeks and then stabilizes. Once the stabilization occurs, it may last from one to two years before recovery.
  • Persistent muscle weakness, muscle inflammation, and rash During this phase, symptoms include persistent muscle weakness, active inflammation of the muscles, and rash lasting up to two years.
  • Recovery Recovery may occur without any apparent consequences, or may occur after two or more years with residual muscle weakness, wasting, or a condition in which muscles in a particular location may become permanently paralyzed as a result of lack of use.

  • Treatment:
    JDMS cannot be cured. However, with supportive therapy and a multidisciplinary team approach to treatment, remission of the disease may be achievable in time. Treatment may include:

  • medications such as glucocorticosteroids and methotrexate and hydroxychloroquine
  • physical and occupational therapy
  • liberal use of sunscreens
  • nutritional support

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