• Juvenile Dermatomyositis

    (JDM)

    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
    IMAGE
    Copyright © Nucleus Medical Media, Inc.

    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
  • LiveWell personal health survey

    How healthy are you really? Find out – free.Learn more

    It's time to stop guessing. If you want to make some changes but just aren't sure how, the free personal health survey from LiveWell is a great place to start.

  • HeartSHAPE Spotlight

    At risk for a heart attack? Learn more

    Fight heart disease and prevent heart attacks. HeartSHAPE® is a painless, non-invasive test that checks pictures of your heart for early-stage coronary disease.

  • Calories and Energy Needs

    Calorie NeedsLearn more

    How many calories do you need to eat each day to maintain your weight and fuel your physical activity? Enter a few of your stats into this calculator to find out.

  • Ideal Body Weight

    Ideal Body WeightLearn more

    Using body mass index as a reference, this calculator determines your ideal body weight range. All you need to do is enter your height.

  • Body Mass Index

    Body Mass IndexLearn more

    This tool considers your height and weight to assess your weight status.


  • Can we help answer your questions?

    Wellmont Nurse Connection is your resource for valuable health information any time, 24 hours a day, seven days a week. Speak to a Nurse any time, day or night, at (423) 723-6877 or toll-free at 1-877-230-NURSE.