Juvenile Dermatomyositis
Juvenile dermatomyositis is one of the conditions in a group of conditions called the
dermatomyositis/polymyositis complex. The conditions in this complex are characterized by
muscle damage due to an inflammatory process of the blood vessels that lie under the skin
and muscles. Skin changes around the eyelids and over the knuckles and finger joints are
also seen. Juvenile dermatomyositis is the condition most often seen in children.
Juvenile dermatomyositis occurs relatively infrequently, but more often in girls about
the age of 7.
What causes juvenile dermatomyositis?
The cause of juvenile dermatomyositis has not yet been determined. However, factors
which are thought to be associated with dermatomyositis include dysfunction of the immune
system, resulting in infections.
What are the symptoms of juvenile dermatomyositis?
The symptoms of juvenile dermatomyositis often appear gradually. At times, there may be
a more acute, or intense, onset of symptoms. The following are the most common symptoms of
juvenile dermatomyositis. However, each child may experience symptoms differently.
Symptoms may include:
- fever
- rash around the eyelids and/or knuckles and finger joints; a rash may also occur on the
elbows, knees, and ankles
- muscle weakness
- fatigue
- malaise
- muscle pain and tenderness
- irritability
- weight loss (due to difficulty swallowing)
- joint pain and inflammation
- calcium deposits under the skin (calcinosis)
- mouth ulcers
Symptoms of juvenile dermatomyositis may resemble other medical conditions or problems.
Always consult your child's physician for a diagnosis.
Phases of juvenile dermatomyositis:
The course of juvenile dermatomyositis is often divided into four phases, based on
symptoms and findings on examination. The four phases are briefly described below:
- Prodromal period
This phase can last for weeks or months. The symptoms seen during this period are
nonspecific (general symptoms which can indicate many different conditions).
- 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 from one to two years before recovery.
- Persistent muscle weakness, muscle inflammation, and rash
During this phase, symptoms include persistent muscle weakness, active inflammation of the
muscles, and rash lasting up to two years.
- Recovery
Recovery may occur without any apparent consequences, or may occur after two or more years
with residual muscle weakness, atrophy (wasting), or contractures (a condition in which
muscles in a particular location may become permanently paralyzed as a result of lack of
use).
How is juvenile dermatomyositis diagnosed?
In addition to a complete medical history and physical examination, diagnostic
procedures for juvenile dermatomyositismay include:
- laboratory tests - 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 - removal of a small piece of muscle for microscopic examination.
- x-rays - a diagnostic test which 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.
Treatment for juvenile dermatomyositis:
Juvenile dermatomyositis cannot be cured. However, with supportive therapy and a
multidisciplinary team approach to treatment, remission of the disease may be achievable
in time. Treatment may include:
- medications such as glucocorticosteroids and methotrexate (to treat the inflammatory
process) and hydroxychloroquine (to help treat the skin disease of dermatomyositis)
- 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)
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