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Definition

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

Causes

The exact cause of JDM is unknown but it is believed to be a problem with the immune system. These problems may cause inflammation of muscle cells and blood vessels that can lead to damage.

Risk Factors

JDM is more common in girls, children living in North American, and children of African American descent. Children with a family history of type 1 diabetes and systemic lupus erythematous are also at an increased risk.

Symptoms

The first JDM symptoms include:

  • Fever
  • Fatigue
  • Lack of appetite
  • Weight loss

As JDM progresses, symptoms may include:

  • Skin changes, such as:
    • Violet-colored, bumpy, or scaly skin rash that occurs on the face, eyelids, knuckles, elbows, knees, chest, and back
    • Skin ulcers
  • Muscle problems, such as:
    • Muscle weakness, especially in the muscles closest to the trunk
    • Muscle pain
    • Problems swallowing and speaking
    • Difficulty moving from a seated to a standing position
    • Frequent falls
  • Sore throat
  • Abdominal pain
  • Shortness of breath
Skin Ulcer
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Diagnosis

The doctor will ask about symptoms and medical history. A physical exam will be done, paying special attention to the skin and muscles. The appearance of a rash may help with diagnosis.

Your child’s blood and urine will be tested to look for changes in certain enzymes and indicators of inflammation.

Inflammation of the muscle may also be confirmed through:

  • 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, although some children may enter a period where symptoms lessen or disappear. Treatment will focus on managing your child’s symptoms. Talk with the doctor about the best treatment plan for your child. Options include:

Medications

Mediations will be given to help manage symptoms. These may include:

  • Corticosteroids, methotrexate, and/or cyclosporine to reduce inflammation and suppress the immune system
  • Mycophenolate mofetil to suppress the immune system in severe cases
  • Hydroxychloroquine to relieve rash symptoms

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

Therapy

Physical activity is an important part of treatment once inflammation is under control. Physical therapy will help your child:

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

Speech therapy can help teach how to cope with swallowing difficulties. A dietitian may also be advised to help with safe food selection and meal planning to promote good nutrition.

Skin Protection

Skin protection is needed to control the rash and skin ulcers. This may include:

  • Avoiding too much time in the sun
  • Wearing a long-sleeved shirt and long pants, a wide-brim hat, and sunglasses
  • Using sunscreens with a sun protection factor (SPF) of at least 30
  • Avoiding exposure during the peak hours of the day

Prevention

There is no method to prevent JDM, since the exact cause of JDM is unknown.

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  http://www.hc-sc.gc.ca  

References

Dermatomyositis. EBSCO DynaMed Plus website. Available at:  https://www.dynamed.com/topics/dmp~AN~T116942/Dermatomyositis . Updated April 11, 2016. Accessed January 23, 2017.

Dermatomyositis (juvenile). American College of Rheumatology website. Available at: http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Dermatomyositis-Juvenile. Published 2015. Accessed January 23, 2017.

Juvenile dermatomyositis. Arthritis Foundation website. Available at: http://www.arthritis.org/about-arthritis/types/juvenile-dermatomyositis-jd/. Accessed January 23, 2017.

Juvenile dermatomyositis. Genetic and Rare Diseases Information Center website. Available at: https://rarediseases.info.nih.gov/diseases/6805/juvenile-dermatomyositis. Accessed January 23, 2017.

Juvenile dermatomyositis. Stanford Children’s Health website. Available at: http://www.stanfordchildrens.org/en/topic/default?id=juvenile-dermatomyositis-90-P01714. Accessed January 23, 2017.

Juvenile dermatomyositis (JDM). Cincinnati Children’s Hospital website. Available at: https://www.cincinnatichildrens.org/health/j/jdms. Updated March 2016. Accessed January 23, 2017.

Juvenile dermatomyositis in children. Boston Children’s Hospital website. http://www.childrenshospital.org/conditions-and-treatments/conditions/j/juvenile-dermatomyositis/overview. Published 2011. Accessed January 23, 2017.

Revision Information

  • Reviewer: EBSCO Medical Review Board Kari Kassir, MD
  • Review Date: 01/2017
  • Update Date: 09/20/2017