Martini A, Ravelli A, Aramini L, Ramenghi B
Clinica Pediatrica, Università di Pavia, IRCCS S Matteo, Italia.
Pediatr Med Chir. 1990 Nov-Dec;12(6):587-91.
Juvenile dermatomyositis (JDM) is a chronic multisystem inflammatory disease that primarily involves skin and muscles. The clinical picture is mainly characterized by the typical cutaneous rash and by skeletal muscle weakness. Nevertheless the vasculitic process may extend in some cases to other organs such as lung, joints or gastrointestinal system. Calcinosis represents a relatively frequent complication of JDM and occurs in most cases during the late stage of the disease. Usually serum levels of muscle enzymes are elevated and electromyography shows signs of myositis. Muscle biopsy is generally performed only in doubtful cases. JDM should be differentiated from the muscle involvement of other diffuse inflammatory connective tissue diseases and from non inflammatory myopathies. The treatment is mainly based on steroids, whose dosage and regimen depend on disease severity. Steroid therapy has greatly improved the prognosis of JDM.
幼年皮肌炎（JDM）是一种主要累及皮肤和肌肉的慢性多系统炎症性疾病。临床表现主要以典型的皮肤皮疹和骨骼肌无力为特征。然而，在某些情况下，血管炎过程可能会扩展到其他器官，如肺、关节或胃肠道系统。钙质沉着是JDM相对常见的并发症，大多数情况下发生在疾病晚期。通常肌肉酶的血清水平会升高，肌电图显示有肌炎迹象。肌肉活检一般仅在疑难病例中进行。JDM应与其他弥漫性炎症性结缔组织病的肌肉受累以及非炎症性肌病相鉴别。治疗主要基于类固醇，其剂量和疗程取决于疾病的严重程度。类固醇疗法极大地改善了JDM的预后。
