What to Know If Your Child Has Juvenile Dermatomyositis

JDM is a chronic condition in children. It occurs when the immune system doesn’t work as it should. This is also known as an autoimmune disease.

In JDM, the immune system attacks muscle and skin cells as well as blood vessels. This causes inflammation that results in a child’s symptoms.

JDM is a very rare condition. It affects just 3,000 to 5,000 children in the United States, or 3 in 1 million children. Doctors generally diagnose the condition in children who are elementary school aged or young teenagers.

Similar conditions, like juvenile myositis and juvenile polymyositis, do not affect the skin.

JDM can progress over time. At first, a child may have mild signs and symptoms that worsen over time. JDM that goes untreated or does not respond to treatment may become very severe and life threatening.

JDM can appear in a number of ways. You may notice a skin rash first. The condition may then affect the muscles and cause other symptoms. The onset of JDM can occur rapidly. Symptoms can also come and go.

Skin rash

This will appear red or purple. It could be very faint. You may notice it on your child’s:

• eyelids
• cheeks (may look like a sunburn)
• fingernails (may look pink)
• knuckles (may look scaly)
• elbows (may look scaly)
• knees (may look scaly)
• ankles
• trunk

Muscle weakness

Following a skin rash, you may notice the following issues related to your child’s muscles:

• weakness at the torso or at parts of the body near it, including the neck, shoulders, hips, and thighs
• problems getting up off the floor or out of a seat
• difficulty climbing stairs
• falls
• difficulty swallowing
• weakening voice
• gastrointestinal (GI) upset
• walking on tip toes if ankles are affected

These symptoms may begin within days of noticing a skin rash or many months after it appears.

Usually, JDM affects the muscles on both sides of the body.

Other symptoms

While skin rash and muscle weakness are primary symptoms of JDM, you may notice others, too, like:

• fatigue that causes a child to tire easily or have a general lack of energy
• fever
• vasculitis
• joint or muscle pain
• limited range of motion in joints because of shortened muscles
• tender muscles
• weight loss
• hard, white lumps under the skin near elbows, knees, and knuckles
• stomach ulcers
• loss of body fat

There’s no confirmed cause of JDM. However, the condition may be linked to:

• genetics, particularly if your family medical history includes autoimmune diseases
• the immune system and certain viruses that trigger it
• the environment

If your child has symptoms of JDM, bring them to their pediatrician for a checkup and diagnosis. Diagnosing JDM may include:

• a medical history and family medical history
• a physical exam, including looking for skin rashes, muscle weakness, and changes to the blood vessels around the nails
• laboratory blood tests that measure muscle enzymes and certain antibodies
• imaging tests, like an MRI, to observe muscle swelling
• a biopsy to examine a small piece of muscle

These tests will allow your child’s doctor to get a full view of the condition. Understanding the severity of it and the way it is affecting your child’s body will help them create a treatment plan.

Treatment for JDM can range from person to person. The goals of treatment are to stop the progression of JDM by controlling or reducing muscle inflammation and improving muscle movement and strength.

The condition is chronic, but treatments can improve the quality of life and may even lead to the end of symptoms, called remission.

Treatment team

Your child’s primary care doctor may add specialists to the treatment plan, such as a:

• rheumatologist
• dermatologist
• physical therapist
• registered dietitian
• speech therapist

Medications

There are a wide number of medications that may help JDM, such as:

• corticosteroids that reduce swelling like prednisone, which should be used for a short time because of harmful side effects
• other medications that suppress the immune system but are less harmful than steroids in the long term, such as:
  • methotrexate
  • hydroxychloroquine
  • anti-TNF drugs
  • rituximab
• intravenous immunoglobulin (IVIG)

Lifestyle

Doctors also recommend making the following lifestyle modifications if your child has JDM:

• Sun protection: Use sunscreen and wear sun-protective clothing, like hats, long-sleeve shirts, and pants.
• Diet: Eat a nutritious and balanced diet that accounts for any difficulties with swallowing.
• Exercise: Find movement your child enjoys to keep their body functioning well, but that also accounts for any mobility limitations.
• Stress management: Try meditation, mindfulness, or deep breathing to reduce stress.

One 2019 study described life with JDM as a “rollercoaster.” Researchers found that children with JDM said they felt:

• different
• sick
• steroidal
• scared
• uncertain

These feelings are related to their symptoms, treatment, and general life as a child. Make sure to consider your child’s emotional needs during treatment. Their pediatrician can connect you with mental health support if needed.

Risk factors for JDM include:

• having family members with autoimmune conditions
• being female (the condition affects twice as many girls as boys)
• having other conditions like diabetes, celiac disease, or arthritis

Although JDM is not curable, treatments can manage symptoms and prevent them from getting worse. A child may even experience remission from symptoms with the right treatment and lifestyle.

There are times when treatments do not prevent the progression of symptoms. JDM can lead to permanent disability or death. This is why it is very important to seek treatment right away.

JDM is a rare, chronic condition that affects the skin, muscles, and blood vessels in a child. There are many treatments available to manage symptoms.

You will need to rely on a medical team for treatment as well as adopt certain habits that keep your child’s body well nourished and strong.