Dermatomyositis (Juvenile): Symptoms, Treatment

Overview

Juvenile dermatomyositis (JDM) is an inflammatory disorder that affects the muscle, skin or blood vessels of children. It is also known as inflammatory myopathy. This disease can cause muscle weakness and skin rashes in kids, because it leads to inflammation and swelling of the muscles and blood vessels under the skin.

In the United States, JDM affects about 2-3 in one million children every year. Most cases occur in kids aging 5-10. Girls are about twice more likely to have the disorder than boys. All races of people can be affected in the US. In the UK, however, chances of getting JDM differ among ethnic groups.

 

Causes

The underlying cause of JDM is unclear. Some believe that it is most likely a hereditary disease, which is the same as other inflammatory disease. The disease is triggered by the immune system’s wrong attack of muscle cells and blood vessels, resulting in inflammation and damage. Your risk of getting the disease increases when you have immunizations, infections, injuries, or sunburn.

 

Symptoms

Two major manifestations of JDM include:

  • Muscle weakness due to inflammation or damage.

The weakness may be progressive. It often occurs in the muscles around the trunk, such as shoulders, hips, neck and upper arms. Signs of muscle weakness involve fatigue and clumsiness. You may also feel difficult to climb stairs, get out of the chair or lift objects. You may fall without any reason.

  • Skin rash.

The rash is usually violet or dusky red in color. It is most commonly seen on the face, eyelids, hands and sometimes the skin above joints. Skin rash is often the first symptom of JDM, but it may be misdiagnosed as allergies or eczema.

Other occasional signs and symptoms of JDM are:

  • Problems in swallowing
  • Irritability
  • Weight loss
  • Mouth ulcers
  • Weak voice
  • Fever
  • Lung problems
  • Intensive stomach pain

 

Diagnosis

If you find skin rash or muscle weakness in your child, please take him or her to the doctor for diagnosis. Common tests used to diagnose JDM include:

  • Magnetic resonance imaging (MRI).

MRI can show the clear image of the affected areas to reveal the inflammation and swelling in the muscles.

  • Blood tests.

Blood tests can detect the proteins associated with inflammation in the blood. Theses proteins can be the autoantibodies related to JDM.

  • Muscle biopsy.

Doctors will take a small piece of the affected muscle tissue and analyze it under the microscope.

  • Electromyography (EMG).

EMG can measure the electric activity of the muscles and find the infected area.

  • Nailfold capillaroscopy.

In this test, doctors will place a bit of oil onto the nail and then look at it with a lighted magnifying glass. Nailfold capillaroscopy can show swelling and distortion of the blood vessels to help with diagnosis.

 

Treatment

Treatment options for JDM mainly focus on reducing inflammation, improving muscle function and preventing disability. Medications that can be used include:

This kind of anti-inflammatory drugs are very helpful in the early treatments. They can help reduce inflammation in the muscle and skin quickly, but long-term use will cause side effects. For example, they will affect your child’s bone growth and eyesight.

Doctors will also suggest methotrexate, a kind of anti-rheumatic drug. It works more slowly than corticosteroids. Patients can take methotrexate along with corticosteroids.

  • Non-steroid medications.

They include IV immunoglobulin, azathioprine, tacrolimus, hydroxychloroquine, and mycophenolate mofetil. If the doctors prescribe these medications for your child, please follow their advice carefully.

Apart from medications, other treatment options are:

  • Physical therapy.

Your child can learn some stretching exercise from the therapist to improve muscle strength.

  • Speech therapy.

If your child develops symptoms of weak voice and talking difficulty, speech therapy would help.

  • Diet assessment.

When your child has difficulty in chewing or swallowing, you should learn to prepare some foods that are easy and safe to eat.

  • Skin protection.

Protecting your child from the sunlight can help control skin rash and muscle weakness. You can use sunscreen, hats with wide brims and sun-proof clothing.

 

Keywords: juvenile dermatomyositis; JDM.

 

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