Juvenile Dermatomyositis (JDM)

What is juvenile dermatomyositis (JDM)?

Juvenile dermatomyositis (JDM) is a rare subset of dermatomyositis that is characterized by inflammation of the muscle (myositis), skin, and blood vessels. Sometimes, remission of JDM is possible, but some children experience a more chronic course of the disease that is less responsive to therapy. Most causes of JDM begin in patients ages 5-10, but also in adults ages 40-50. Interestingly, girls are affected twice as often as boys.

What causes juvenile dermatomyositis (JDM)?

Dermatomyositis is involved in a group of muscle disorders called inflammatory myopathies, the causes of which are unknown. Even so, some doctors and researchers speculate that the body’s immune system mistakenly directs inflammation against the muscle cells and blood vessels, causing the characteristics of dermatomyositis.

What are the symptoms of juvenile dermatomyositis (JDM)?

Children with JDM have varying symptoms, but the primary symptoms of the disease include muscle weakness and skin rash. The skin rash is violet-colored or dusky red and occurs most commonly on the face, eyelids, and around the nails, elbows, knees, chest, and back. The muscle weakness in JDM is progressive (gradually worsens) and specifically occurs in the muscles closest to the trunk of the body. In addition to these, other common signs and symptoms of JDM include:

• Difficulty swallowing
• Voice changes
• Muscle pain or tenderness
• Fatigue, fever, and weight loss
• Hardened calcium deposits under the skin
• Stomach ulcers and intestinal tears
• Lung problems

How is juvenile dermatomyositis (JDM) diagnosed?

JDM can be diagnosed after a thorough clinical examination and a review of patient and family history. To confirm a diagnosis, the following tests may be used:

• MRI scanning
• Muscle biopsy
• Blood tests
• Nailfold capillaroscopy

What are the available treatments for juvenile dermatomyositis (JDM)?

There is no cure for JDM, so the goals of treatment are to minimize inflammation, improve overall muscle function, and prevent disability. The first treatment choice for many is corticosteroids, such as prednisone or oral glucocorticoids, as they work fast. However, these corticosteroids can have some serious side effects, so medications like methotrexate, immunoglobulin, or other steroid-sparing agents can be administered instead. These have fewer potential side effects, but they work slower. In addition to medications, patients with JDM should protect their skin and attend physical therapy. If the swallowing muscles are affected by the disease, speech therapy and a diet assessment may also be beneficial.

Where can I find more information on juvenile dermatomyositis (JDM)?

• American College of Rheumatology
• Medscape
• Cleveland Clinic