JUVENILE DERMATOMYOSITIS (JDM) | ABP Board Review

JUVENILE DERMATOMYOSITIS (JDM)
• Girls more commonly affected
• Bimodal: 3–7 years of age and early teenage years
• Rash with proximal muscle weakness
– Classic heliotrope (faint purple to red discoloration of the eyelids)
– Nailfold capillaritis
– Seasonal clustering of onset
? infectious triggers

Dermatomyositis
• Gottron papules
• Dystrophic calcification: calcinosis cutis
• Proximal muscle weakness
• Photosensitive rash DDx: SLE vs. JDM
• Skin ulcerations are rare: can portend GI vasculitis - poor prognosis
• Dysphagia, dysphonia can occur
• No increased risk for malignancy in children
• T2 or STIR image MRI of thighs can reveal myositis
• Diagnosis
– Heliotrope rash or Gottron papules
+
– 2 of the following for probable, 3 for definite
diagnosis:
• Symmetric proximal muscle weakness
• ↑ CK, aldolase, LDH, transaminases
• EMG abnormalities
• Abnormal muscle biopsy
• Rx: high-dose steroids 2–3 mg/kg/day (or pulse steroids IV methylprednisolone)
• IVIG (monthly)
• Hydroxychloroquine, MTX, CYA, MMF, IV CYP, combination DMARDs
• Physical therapy
• Sun avoidance
• Refractory cases — rituximab, TNFi therapy

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