Juvenile Dermatomyositis

"Even though juvenile dermatomyositis is rare and can be serious, most children we treat can go into full remission."

--Susan Kim, MD, MMSc, Children's rheumatologist

It’s often not until a rash appears around a child’s eyes or on his knuckles, knees or elbows that parents of children with juvenile dermatomyositis make a doctor’s appointment. Yet your child may have been feeling the effects of the condition for months—and sometimes years—in the form of fevers, fatigue or muscle weakness—before the rash ever breaks out.

Juvenile dermatomyositis (JDM) may often be misdiagnosed because it looks at first to simply be a skin condition. But it’s actually a rare autoimmune disorder in which a child’s immune system attacks blood vessels throughout his body (called vasculopathy), causing muscle inflammation (called myositis).

Here are some other facts you may not have known about JDM:

  • Juvenile dermatomyositis affects more children than adults.
  • The exact cause of JDM isn’t known, but problems with a child’s immune system are believed to be a factor.
  • JDM is rare, affecting only 3,000 to 5,000 kids under age 18 in the United States.
  • There’s no typical age when a child first gets the condition, but the average age is 7 years old.
  • The disease often begins with nonspecific, general symptoms (such as fever, fatigue and muscle weakness).
  • A rash may develop around the eyelids (known as a heliotrope rash), elbows, knees and/or knuckles and finger joints or involve the toes (called Gottrons papules).
  • In mild cases, a child may recover without any apparent long-term effects.
  • Without treatment, a child may have residual muscle weakness, atrophy (wasting of the muscles) or contractures, a condition where children lose normal mobility of their joints.
  • While there’s currently no known cure for, we’ve found that using a combination of immunosuppressive therapies can put the disease into remission over time.

The detailed information on the following pages will help you gain a better understanding of juvenile dermatomyositis and a clearer picture of what to expect.

How Boston Children's Hospital approaches juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is a serious condition for which there is currently no known cure. Here at Children’s, we aim to put your child’s condition into remission by using supportive therapy and a multidisciplinary team approach to treatment. 

  • Though historically it is reported that only a third of patients with JDM go into full remission, we find the majority of our patients can attain remission with treatment.
  • In 2006, Children’s formed the Dermatology-Rheumatology Center to treat kids with conditions like JDM. This partnership allows us to take advantage of our expertise in both areas.
  • At Children’s, we specialize in innovative, family-centered care. From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s medical, emotional and psychosocial needs. Our interdisciplinary team offers your family many avenues of support, including the services of social workers, Child Life specialists and psychiatric nurses.

Juvenile dermatomyositis: Reviewed by
Susan Kim, MD, MMSc © Children’s Hospital Boston; posted in 2011