Dermatomyositis (Juvenile)


Juvenile dermatomyositis (JDM) is a disease in which a child’s immune system primarily attacks the skin and muscles or blood vessels. The cause of JDM remains unknown to date but has been linked to genetics and environmental factors that lead to the disease. JDM often affects large muscles around the neck, shoulders, and hips, causing primarily weakness. Children with JDM may struggle to do basic movements such as climbing stairs, getting in and out of a car or chair, or brushing their hair. JDM affects three in one million children each year, usually between ages 5-10.

What Are the Signs/Symptoms?

The characteristic rash is violet or dusky red and may be seen on the eyelids, nails, elbows, knees, chest, and back. Knuckle rashes may be mistaken for eczema. Other signs may include difficulty swallowing, voice changes, tender or painful muscles, fatigue, fever, weight loss, hard calcium deposits under the skin, stomach ulcers, intestinal tears, and lung problems. Blood tests often show evidence of muscle damage and JDM-related autoantibodies. The diagnosis is supported by an MRI and a muscle biopsy, both of which show signs of inflammation.

What Are Common Treatments?

The treatment goals are to eliminate inflammation, restore and preserve muscle performance, and prevent disability. High-dose intravenous (injection into a vein) or oral corticosteroids are first-line treatments. Symptoms and inflammation measures may start to improve in 2–4 weeks with use. However, long-term corticosteroid use can cause many side effects. Methotrexate is often a first-line therapy used in combination with steroids. Alternative treatment options include intravenous immunoglobulin, azathioprine, cyclosporine, tacrolimus, rituximab, mycophenolate mofetil, and anti-TNF biologics, which are often used if the first-line treatment fails or is not tolerated.

Hydroxychloroquine and topical steroid creams are drugs used to help treat the skin component of the disease. JDM is unique among most pediatric rheumatic diseases in that it can often be cured if treated aggressively at the beginning.

Living with Juvenile Dermatomyositis

Physical therapy can help children with JDM build their strength and prevent muscle wasting and stiffness. Children with JDM need to protect themselves from the sun by wearing sunscreen, hats with brims, and protective clothing when playing outdoors. If neck or throat muscles are affected, the child may need speech therapy. A dietitian can create a diet for children with chewing or swallowing problems. They should eat a healthy, balanced diet and stay active.

Updated February 2023 by David Waldburg, MD, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.

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