Juvenile Dermatomyositis

Juvenile Dermatomyositis

(JDM)

Derr-MAA-toe-MY-o-SI-tiss

Definition

Juvenile dermatomyositis (JDM) is a rare disease of the skin, muscles, and blood vessels.

Causes

The exact cause of JDM is not known. It is thought to be a problem with the immune system. These problems may cause swelling of muscle cells and blood vessels that can lead to damage.

Risk Factors

JDM is more common in girls, children living in North America, and children who are Black. Children with a family history of type 1 diabetes and lupus are also at a higher risk.

Symptoms

The first JDM symptoms are:

  • Fever
  • Fatigue
  • Lack of hunger
  • Weight loss

As JDM gets worse, symptoms may be:

  • Skin changes, such as:
    • Violet-colored, bumpy, or scaly skin rash on the face, eyelids, knuckles, elbows, knees, chest, and back
    • Skin sores
  • Muscle problems, such as:
    • Weakness, especially in the muscles closest to the trunk
    • Pain
    • Problems swallowing and speaking
    • Problems moving from a seated to a standing position
    • Falling often
  • Sore throat
  • Belly pain
  • Shortness of breath

Skin Ulcer

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Diagnosis

You will be asked about your symptoms and health history. A physical exam will be done. It will focus on your skin and muscles. The appearance of a rash may help with diagnosis.

Your child’s blood and urine will be tested. This will look for changes in some enzymes and signs of swelling.

Your muscles may be tested using:

  • MRI
  • Biopsy of your child’s muscle

Electromyography is an electrical test that can find nerve or muscle damage.

Treatment

There is no cure for JDM. Some children may have times when symptoms lessen or go away. Treatment will focus on managing symptoms. Talk with the doctor about the best plan for your child. Choices are:

Medications

Medicine will be given to help ease symptoms. These may be:

  • Corticosteroids, methotrexate, or cyclosporine to ease inflammation and suppress the immune system
  • Mycophenolate mofetil to suppress the immune system in people with severe symptoms
  • Hydroxychloroquine to ease a rash

IV immunoglobin (IVIG) may be given to slow down the inflammatory process.

Therapy

Physical activity can help once inflammation is under control. Your child will:

  • Maintain and improve strength and flexibility
  • Prevent muscle wasting and stiffness
  • Choose the right activities for exercising regularly

Speech therapy can help teach how to cope with problems swallowing. A dietitian may also help you and your child pick safe foods and plan meals.

Skin Protection

Skin protection is needed to control the rash and skin sores:

  • Do not spend too much time in the sun.
  • Wear a long-sleeved shirt and long pants, a wide-brim hat, and sunglasses.
  • Use sunscreens with an SPF of at least 30.
  • Stay out of the sun during the peak hours of the day.

Prevention

There is no way to prevent JDM. The cause is not known.

RESOURCES:

American College of Rheumatology
http://www.rheumatology.org
Healthy Children—American Academy of Pediatrics
http://www.healthychildren.org

CANADIAN RESOURCES:

Canadian Rheumatology Association
https://rheum.ca

References:

Dermatomyositis. EBSCO DynaMed Plus website. Available at: https://www.dynamed.com/topics/dmp~AN~T116942/Dermatomyositis. Updated June 4, 2018. Accessed May 2, 2019.
Dermatomyositis (juvenile). American College of Rheumatology website. Available at:
...(Click grey area to select URL)
Updated March 2019. Accessed May 2, 2019.
Enders FB, Bader-Meunier B, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis. 2017 Feb;76(2):329-340.
Juvenile dermatomyositis. Arthritis Foundation website. Available at:
...(Click grey area to select URL)
Accessed May 2, 2019.
Juvenile dermatomyositis. Stanford Children’s Health website. Available at:
...(Click grey area to select URL)
Accessed May 2, 2019.
Last reviewed March 2019 by EBSCO Medical Review Board Kari Kassir, MD
Last Updated: 5/2/2019

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