Juvenile dermatomyositis
Overview
A very rare autoimmune disorder where the body's own immune system attacks blood vessels and causes them to become inflamed. The condition is characterized by progressive muscle weakness and a characteristic pinkish-purple rash.
Symptoms
- Muscle weakness
- Chronic muscle inflammation
- Skin rash
- Difficulty swallowing
- Muscle pain
- Tender muscles
- Weight loss
- Fever
- Fatigue
- Nasal voice
- Falling
- Contractures
- Abdominal pain
- Clumsiness
- Patchy skin rash
- Pinkish-purple discolored skin rash
- Light sensitivity
- Hard calcified lumps under skin
- Hard calcified lumps in muscles
Causes
The underlying cause of JDM is unknown. It most likely has a genetic component, as other auto-immune disease tend to run in the families of patients. The disease is usually triggered by a condition that causes immune system activity that does not stop as it should, but the trigger is almost certainly not the cause in most cases. Common triggers include immunizations, infections, injuries, and sunburn.
Diagnosis
JDM is diagnosed by a combination of patient/parent observations, clinical examination, and laboratory blood tests.
Prognosis
Prognosis of Juvenile dermatomyositis: The rate of progression is variable but muscle symptoms tend to occur within weeks or months of the skin rash appearing. Steroid treatment is usually very effective.
Treatment
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)