Juvenile Dermatomyositis

Juvenile dermatomyositis (JDM) can seriously impact your child’s daily life, and can also tax the emotional health of your family. Since it’s so rare, juvenile dermatomyositis (JDM) can seem scary to family and child—and often it’s puzzling to pediatricians, who may not be familiar with the condition. But within the Rheumatology Program at Children’s Hospital Boston, JDM is actually the most common condition we see after arthritis. And the good news is that with proper treatment, it can be well managed.

The information on the following pages is a good starting point to help you learn more about JAD and to give you and your family a general idea of what to expect in the weeks and months ahead.

What is juvenile dermatomyositis?

Juvenile dermatomyositis is a rare autoimmune disorder in which the immune system attacks blood vessels throughout the body (called vasculopathy), causing muscle inflammation (known as myositis). As a result, your child may be exhausted by simple, everyday activities, like walking up stairs or lifting a backpack, or he may have trouble keeping up with friends at recess or on the soccer field. Often it is not until the condition appears in the form of a rash around the eyelids or over the knuckles or finger joints, that parents seek medical attention.

How common is juvenile dermatomyositis?

Juvenile dermatomyositis is rare, affecting only 3,000 to 5,000 children in the United States under age 18.

Are there any significant side effects to juvenile dermatomyositis?

Juvenile dermatomyositis can result in calcinosis, which are calcium deposits under the skin, around the joints and in the intramuscular facial planes. Mild cases of calcinosis are usually cleared with treatment, but in some cases, calcinosis can cause chronic severe pain, joint contractures (a condition in which muscles in a particular location may become permanently paralyzed), fever and other complications.

Here at Children’s, we have conducted numerous studies of the formation and treatment of calcinosis—specifically early, aggressive treatment—with the goal of uncovering the most effective way to manage it.

What are the different phases of juvenile dermatomyositis?

The course of juvenile dermatomyositis has historically been divided into four phases:

• Prodromal period: This phase can last for weeks or months. JDM is difficult to diagnose in this phase because the symptoms are so nonspecific.

• Progressive muscle weakness and rash: In this phase, muscle weakness increases for days and weeks and then stabilizes. Once the stabilization occurs, it may last for several years before a child fully recovers.

• Persistent muscle weakness, muscle inflammation and rash: During this phase, symptoms include persistent muscle weakness, active inflammation of the muscles and a rash lasting up to two years.

• Recovery: Recovery may occur after two or more years with residual muscle weakness, atrophy (wasting of the muscles), or contractures—a condition in which muscles in a particular location may become permanently paralyzed. Other times, a child recovers without any apparent long-term effects.

Causes

What causes juvenile dermatomyositis?

Doctors don’t fully understand what causes juvenile dermatomyositis. However, we think that certain children have a genetic predisposition to it. Factors thought to be associated with the condition include problems with the immune system that are triggered by environmental factors (which can include an illness and even sunlight).

What we do know is that a number of factors go into a child’s development of juvenile dermatomyositis, so it’s not caused by just one thing.

Signs and symptoms

What are the symptoms of juvenile dermatomyositis?

The symptoms of juvenile dermatomyositis often appear gradually, although your child may have more acute, or intense, symptoms. Each child may experience symptoms differently, but the most common signs are:

•  fever
• rash around the eyelids (known as heliotrope rash) and/or knuckles and finger joints (called Gottren’s papules); a rash may also occur on the elbows, knees and ankles
• muscle weakness
• fatigue
•  malaise
•  muscle pain and tenderness
•  irritability
• weight loss
•  joint pain and inflammation
•  calcium deposits under the skin (called calcinosis)
• mouth ulcers

Symptoms may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.

FAQ

Q: As a mother, did I cause my child’s juvenile dermatomyositis (JDM) somehow during pregnancy?

A: No. JDM is never caused by something a mother did or didn’t do. We are still unsure what causes JDM, but suspect that there’s a genetic predisposition. If there’s a history of autoimmune conditions in your family, you’re more likely to have a child with an autoimmune disorder. However, in the case of JDM, it also looks as if the condition is brought on by one or more environmental triggers, which can include an illness and even sunlight (which damages the skin).

Q: Could vaccines have caused my child’s juvenile dermatomyositis?

A: A vaccine may trigger your child’s JDM if he was already predisposed to getting the condition, but no one particular vaccine is to blame. In addition, the development of JDM is likely due to more than one environmental trigger—not just a vaccine.

Q: If I have another child, will he or she also develop JDM?

A: The answer is most likely not, although your other children may be at a higher risk of developing some sort of autoimmune disorder. According to studies, there is a 30 percent concordance rate of JDM between identical twins.

Q: Should I give my child a special diet to help with her JDM?

A:No existing data suggests that JDM can be fixed with any sort of special diet—organic, gluten-free or otherwise—or that a certain diet might alleviate your child’s symptoms. We do suggest that patients follow a very balanced diet with enough calcium and vitamin D.

Q: Can my child still play sports?

A: Some children with JDM experience muscle weakness severe enough to prevent them from participating in rigorous physical activity. Whether your child will be affected will depend on how quickly she is treated and how well she responds to treatment. In all cases, we do suggest practicing sun safety, so be sure that your child wears adequate SPF and sun protective clothing whenever there will be sun exposure—especially if she’s playing outdoor sports.

Q: Will my child ever look normal again?

A: Your child may come to us with a rash or other skin manifestations of JDM. However, with treatment, most skin manifestations clear up, and your child will look and act like any other child his age.

Q: Is there a cure for juvenile dermatomyositis?

A: Technically there isn’t a cure for JDM, but with our treatment regimens, we aim to get your child into full remission and off medicines. Most children with autoimmune conditions like JDM will need to see a rheumatologist regularly for review. Once in remission, most kids come back to Children’s or visit a rheumatologist in their area at least once every year.

Q: What should I expect when it comes to my child’s quality of life?

A: This is a question unique to each family. The Children’s Hospital Boston interdisciplinary team welcomes the opportunity to discuss any of your concerns. We expect the vast majority of children will be able to live a full and active life, engaging in all (or most) normal childhood activities. Your child’s experience should be much the same as any other kid’s.

How is juvenile dermatomyositis diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for juvenile dermatomyositis may include:

• laboratory tests, used to assess the presence of antibodies, muscle enzymes and indicators of inflammation in the blood

• electromyography (EMG), an electrical test to determine nerve or muscle damage

• muscle biopsy, the removal of a small piece of muscle for microscopic examination

• x-rays, a diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film

• magnetic resonance imaging (MRI), a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body

If your child is being treated for juvenile dermatomyositis (JDM) at Boston Children's Hospital, your entire family is involved. As an interdisciplinary team, we partner closely with families to understand and address any individual, family or school issues which may arise as we work together to optimize your child's adjustment to the challenges of living with JDM.

If your child has JDM, there are three possible outcomes:

• continuous disease
• chronic limitations
•  full remission

Here at Children's, we have extensive experience in treating juvenile dermatomyositis. Within our Rheumatology Program, JDM is actually the most common condition we see (second only to arthritis). Compared to other institutions, which report patients in full remission at about 33 percent, nearly 90 percent of the kids treated at Children's will wind up in full remission.

While there's currently no known cure for juvenile dermatomyositis, we've found that using a combination of immunosuppressive therapies can put the disease into remission over time, especially if we work to get the condition under control as early as possible.  We aim to get your child into full remission and off medicines over time.

We tailor treatment for your child's juvenile dermatomyositis to the stage of his condition and his body's initial response to treatment. Prescribed treatment methods may include:

• medications, such as glucocorticosteroids and methotrexate, which treat the inflammatory disorder, and hydroxychloroquine, which treats the skin-related aspect of the condition (dermatomyositis)

• intravenous therapy

• physical and occupational therapy to improve muscle function and strength

• liberal use of sunscreens to prevent further irritation or damage to the skin

• nutritional support to ensure an adequate diet

• alternative therapies, such as reiki, acupuncture and massage in order to make your child more comfortable

Will my child's skin return to normal?

If your child has a visible rash or other skin problems relating to JDM, you may be wondering, “Will my child look normal again?” Fortunately, with treatment, most skin problems clear up, and your child will look and act like any other child his age.

What are the expectations regarding my child's quality of life?

This is a question unique to each family, and our team welcomes the opportunity to discuss any of your concerns. We expect vast majority of children will be able to live a full and active life, engaging in all (or most) childhood activities. Your child's experience should be much the same as any other kid his or her age.

What is the long-term outlook for my child?

It's important to keep in mind that most kids with JDM need to see a rheumatologist regularly. Most children come back to Children's or visit a rheumatologist in their area at least once every other year once they are in remission.

We place great emphasis on ongoing care and we will work with you to establish a relationship with a rheumatologist in your area. The Center for Adults with Pediatric Rheumatic Illness (CAPRI) is a collaborative effort between our hospital and Brigham and Women's Hospital. CAPRI is devoted to the multidisciplinary treatment of adults with childhood-onset arthritic and inflammatory disorders. Specialists from both hospitals collaborate as we transition patients into CAPRI when they reach adulthood, allowing them to see some of the same doctors as they did when they were children. 

Researchers in our Rheumatology Program are looking at the results of aggressive treatment for juvenile dermatomyositis as it affects complete remission.

Between 1994 and 2004, 49 children with JDM received standardized therapy with steroids and methotrexate. If a patient's strength or muscle enzyme levels did not improve with this initial therapy, additional medications were quickly added. We then measured how long it took for a child to reach complete remission.

Our findings suggested that aggressive treatment of JDM aimed at achieving rapid, complete control of muscle weakness and inflammation improves outcomes and reduces disease-related complications. In more than one-half of the children whose disease was treated in this manner (28 of the 49), a prolonged, medication-free remission was attained an average of 38 months from the time of diagnosis. We concluded that aggressive management of juvenile dermatomyositis results in improved outcome and decreased incidence of calcinosis.

Some of our other research projects also include studying:

• aggressive treatment directed at achieving rapid and complete control of muscle inflammation
• the effects of biologic agents on treatment of JDM and other autoimmune conditions.

Learn more about our research.

Clinical trials

Children’s is known worldwide for pioneering some of the most effective diagnostic tools, therapies and preventive approaches in pediatric medicine. A significant part of our success comes from our commitment to research—and to advancing the frontiers of health care by conducting clinical trials.

Children’s coordinates hundreds of clinical trials at any given time. Clinical trials are studies that may involve:

• evaluating the effectiveness of a new drug therapy
• testing a new diagnostic procedure or device
• examining a new treatment method for a particular condition
• taking a closer look at the causes and progression of specific diseases

Children’s is involved in several multi-site clinical trials and studies focusing on pediatric dermatology and rheumatology. While children must meet strict criteria in order to be eligible for a clinical trial, your child may be a candidate for participation in a study. Before considering this option, you should be sure to:

• consult with your child’s treating physician and treatment team
• gather as much information as possible about the specific course of action outlined in the trial
• do your own research about the latest breakthroughs relating to your child’s condition

Taking part in a clinical trial at Children’s is entirely voluntary. Our team will be sure to fully address any questions you may have, and you may remove your child from any medical study at any time.

Search current and upcoming clinical trials at Boston Children’s.

Search the National Institute of Health’s list of clinical trials taking place around the world.