皮肌炎是横纹肌非化脓性炎性肌病,临床表现多样,主要累及皮肤、肌肉、胃肠,多伴有特征性的皮肤损害。儿童皮肌炎有其自身特点,较成人型更易引起软组织钙化,雷诺现象及并恶性肿瘤者少见。目前药物治疗仍以糖皮质激素为首选,合并免疫抑制剂治疗可缩短激素使用时间,减少激素用量。难治型、反复型皮肌炎可选用IVIG、血浆置换等治疗。 Dermatomyositis is a striated muscle non suppurative inflammatory myopathy, clinical manifestations and diverse, mainly involving the skin, muscle, gastrointestinal, often accompanied by characteristic skin lesions. Children’s dermatomyositis has its own characteristics, more likely to cause than the adult type of soft tissue calcification, Renault phenomenon and malignant tumors are rare. At present, drug therapy is still the first choice of glucocorticoid, combined immunosuppressive therapy can shorten the use of hormones, reduce hormone dosage. Refractory, recurrent dermatomyositis can be used IVIG, plasma exchange and other treatment.