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Juvenile Dermatomyositis

  • Juvenile Dermatomyositis (JDM) has a broad range of severity and is a serious disease requiring specialist assessment and management.
  • JDM can present at any age, with a variable presentation and course.
  • Typical features include:
    • Characteristic skin involvement - malar rash, heliotrope rash over the eyelids, photosensitivity, vasculitis, and nail fold capillary changes. Calcinosis (painful or painless lumps or sheets under the skin) are a feature of late diagnosis or poorly controlled disease.
    • Chronic skin disease can result in Gottron's papules over the knuckles, elbows or knees. Skin ulceration may also be present.
    • Proximal muscle weakness can present acutely or indolently with difficulty on stairs or fatigue on walking.
    • Myalgia, arthralgia and arthritis and joint contractures.
    • Systemic symptoms such as malaise, fever, weight loss or anorexia 
  • Bulbar muscle involvement and chest wall muscles weakness may result in risk of aspiration and pneumonia.  
    • Bulbar muscle involvement and chest wall weakness may be suspected if patients have a weak voice, difficulty swallowing, shortness of breath of difficulty coughing.
    • Remember 'dysphagia, dysphonia, dyspnoea'.
  • Interstitial lung disease and gut vasculitis can occur with potential for severe morbidity and mortality.
  • Unlike adult onset DM, JDM does not tend to be associated with malignancy.
    • Routine screening for malignancy in children with JDM is not necessary. 

The photograph below shows subcutaneous calcinosis in JDM

The photograph below shows typical Gottron's papules over the knuckles in JDM

The photograph below shows severe joint contracture and muscle wasting from chronic JDM that has not been treated optimally.

The radiograph below shows severe calcinosis around the knee in chronic JDM