J Rheum Dis.  2022 Jan;29(1):14-21. 10.4078/jrd.2022.29.1.14.

Juvenile Dermatomyositis

• Rhim JW

• KMID: 2523455
• DOI: http://doi.org/10.4078/jrd.2022.29.1.14

Abstract

Juvenile dermatomyositis (JDM) is a systemic capillary vasculopathy. Patients present with proximal muscle weakness, raised muscle enzymes, and pathognomic skin rashes such as heliotrope rash, Gottron’s papules. Main complications are calcinosis, lipodystrophy, osteoporosis. Complement-mediated damage of vessels is a major mechanism. Magnetic resonance imaging is currently widely used to diagnosis of JDM. The goals of treatment are to control inflammatory myositis and prevent disease complication. Early, aggressive treatment of JDM associated with a better prognosis. High-dose corticosteroids in combination with methotrexate is the mainstay of treatment. The course of JDM is variable.

Keyword

Juvenile dermatomyositis; Muscle weakness; Rash

Figure

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  Figure 1 Classification tree for subgroups of idiopathic inflammatory myopathies (IIM). PM: polymyositis, IBM: inclusion body myositis, ADM: amyopathic dermatomyositis, DM: dermatomyositis, JDM: juvenile dermatomyositis, EULAR/ACR: European League Against Rheumatism/American College of Rheumatology. *The PM subset includes immune-mediated necrotizing myopathies (IMNM).

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