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(JDM)

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 harm.

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:
    • Purple, 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 sitting or standing
    • Falls
  • Sore throat
  • Belly pain
  • Shortness of breath
Skin Ulcer
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Diagnosis

You will be asked about your child's symptoms and health history. A physical exam will be done. It will focus on your child's skin and muscles.

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

Your child's muscles may be tested using:

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

Exercise can help when the inflammation is under control. It can:

  • Maintain and improve strength and flexibility
  • Prevent muscle wasting and stiffness

Speech therapy can help teach children how to cope with problems swallowing. A dietitian can also help with meal planning.

Skin Protection

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

  • Time in the sun will need to be limited. Peak hours will need to be avoided.
  • Long-sleeved shirts and long pants, a wide-brim hat, and sunglasses should be worn in the sun.
  • Sunscreen should have an SPF of at least 30.

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 

Health Canada  https://www.canada.ca  

References

Dermatomyositis. EBSCO DynaMed website. Available at:  https://www.dynamed.com/topics/dmp~AN~T116942/Dermatomyositis . Updated June 4, 2018. Accessed July 18, 2019.

Dermatomyositis (juvenile). American College of Rheumatology website. Available at: http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Dermatomyositis-Juvenile. Updated March 2019. Accessed July 18, 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: http://www.arthritis.org/about-arthritis/types/juvenile-dermatomyositis-jd/. Accessed July 18, 2019.

Juvenile dermatomyositis. Stanford Children’s Health website. Available at: http://www.stanfordchildrens.org/en/topic/default?id=juvenile-dermatomyositis-90-P01714. Accessed July 18, 2019.

Revision Information

  • Reviewer: EBSCO Medical Review BoardKari Kassir, MD
  • Review Date: 06/2019
  • Update Date: 07/18/2019