What is Juvenile Dermatomyositis?

Juvenile dermatomyositis is an inflammatory condition that affects children and adolescents. More frequently diagnosed in females, juvenile dermatomyositis is characterized by distinct dermal inflammation and pronounced, impaired muscle function. In the absence of a cure, treatment for this form of dermatomyositis is generally centered on symptom management and slowing disease progression.

Dermatomyositis is commonly classified as a myopathic disorder. The initial cause for dermatomyositis onset in most cases remains elusive. Commonly linked to the presence of infection, this condition involves the overproduction of autoantibodies by the immune system in response to an unknown trigger. As the autoantibodies circulate throughout one’s system, they attack blood vessels within the muscular layers of soft tissue. The constant onslaught of autoantibodies results in the gradual deterioration of muscle tissue.

A diagnosis of juvenile dermatomyositis is generally made based on the presence of the disorder's characteristic dermal inflammation. After an extensive consultation and physical examination is performed, the individual will usually be referred for additional diagnostic testing to confirm the diagnosis. Imaging tests, including magnetic resonance imaging (MRI), are conducted to evaluate the condition of the muscles and identify inflammation. Blood work will generally be checked for markers indicative of abnormalities or infection, including a high white blood cell count and elevated creatine kinase (CK) levels. Additional testing may involve tissue and muscle biopsies to confirm a diagnosis and further evaluate the extent of muscle deterioration that has occurred.

Young people who develop this chronic condition will exhibit a variety of typical signs and symptoms. The most common presentation of juvenile dermatomyositis involves the development of a dark red to almost purple-colored rash that presents on areas that include the face, upper torso and digits. As the disease progresses, individuals will notice a proportional loss of muscle tone and function affecting parallel parts of the body. Additional signs include widespread muscle discomfort, respiratory dysfunction, and pronounced fatigue. Complications associated with this condition often include an increased risk for infection, pneumonia, and calcinosis.

Treatment for juvenile dermatomyositis involves corticosteroidal and antimalarial medications to help alleviate inflammation. Over-the-counter (OTC) analgesic medications may also be used to help ease any localized or widespread muscle discomfort that develops; as the condition progresses, prescription-strength pain relievers may be necessary. Physical therapy is often utilized to help the individual maintain flexibility and strength in his or her muscles for as long as possible. Other treatment options include the intravenous and topical administrations of antibody and immunosuppressive therapies to help inhibit the body’s autoantibody production and immuno-response to chronic inflammation.