TY - CHAP M1 - Book, Section TI - Juvenile Dermatomyositis A1 - Kim, Susan A2 - Zaoutis, Lisa B. A2 - Chiang, Vincent W. Y1 - 2017 N1 - T2 - Comprehensive Pediatric Hospital Medicine, 2e AB - Juvenile dermatomyositis (JDM) is the most common pediatric acquired inflammatory myopathy. This diffuse vasculopathy primarily involves inflammation in skin and striated muscle, which often leads to significant weakness and physical limitations. However, despite its nomenclature JDM is a multisystem disease. Characteristic cutaneous features, which classically include heliotrope rash and Gottron papules, aid in the diagnosis. The etiology of this condition is unknown, and has an estimated incidence of 1 to 3 in 1 million children,1,2 with approximately a 2:1 female predominance. Affected children are typically between 4 and 10 years of age with an average age of onset of 7 years. Outcomes in JDM may vary, but historically there was significant morbidity; without adequate treatment, one-third of affected persons will have moderate to severe disability. Mortality attributable to the disease occurs in one-third of children.3,4 SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1146122423 ER -