Juvenile Dermatomyositis — SCE Dermatology MCQ

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ModeratePaediatric DermatologyJuvenile DermatomyositisSCE Dermatology

A 4-year-old girl has juvenile dermatomyositis. She has heliotrope rash, Gottron papules, and proximal muscle weakness. Compared to adult DM, what feature is MORE common in juvenile DM?

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Correct answer: A — Calcinosis cutis

Calcinosis cutis is significantly more common in juvenile DM (JDM) than adult DM, occurring in 30-70% of JDM patients (versus <10% in adults). Calcinosis in JDM is often extensive and disabling, affecting subcutaneous tissue, fasciae, and muscle. It is associated with delayed diagnosis and inadequate initial treatment. Anti-NXP2 (anti-MJ) antibodies are the myositis-specific antibody most associated with calcinosis in JDM. In contrast to adult DM, malignancy is extremely rare in JDM. Anti-TIF1-gamma in children indicates a DM phenotype different from the adult cancer-associated pattern. Treatment of JDM calcinosis is challenging (Diltiazem, Sodium thiosulfate, Aluminium hydroxide).

Reference: BAD 2016 DM Guidelines; Paediatric Rheumatology