Aihara Y, Mori M, Ibe M, Kuriyama T, Takahashi Y, Shimizu C, Shike H, Mitsuda T, Yokota S
Department of Pediatrics, Yokohama City University, School of Medicine.
Ryumachi. 1994 Oct;34(5):879-84.
Juvenile dermatomyositis (JDM) is a multisystem disease characterized by acute and chronic nonsuppurative inflammation of striated muscle and skin. JDM is classified into an independent entity in the classification of dermatomyositis, and it is marked by the development of calcinosis late in the course of the disease. Since an appropriate steroid therapy for the disease was established, the prognosis of the JDM has been improved except for a fulminant case. Frequency of calcinosis in JDM also has been decreasing because of the adequate steroid therapy. However, once calcinosis universalis occurs, this is the most troublesome and debilitating complication in JDM. Treatment of calcinosis universalis in JDM, so far, remains unsatisfactory. We report here a case of JDM, who suffered from at the age of 3 years, with calcinosis universalis that showed remarkable improvement to orally administrated aluminum hydroxide.
幼年皮肌炎(JDM)是一种多系统疾病,其特征为横纹肌和皮肤的急性和慢性非化脓性炎症。JDM在皮肌炎分类中被归为一个独立的病种,其特点是在疾病后期出现钙质沉着。自从确立了针对该疾病的适当类固醇疗法后,除暴发性病例外,JDM的预后已有所改善。由于类固醇疗法得当,JDM中钙质沉着的发生率也一直在下降。然而,一旦发生全身性钙质沉着,这就是JDM中最麻烦且使人衰弱的并发症。迄今为止,JDM中全身性钙质沉着的治疗仍不尽人意。我们在此报告一例3岁时患JDM并伴有全身性钙质沉着的病例,该病例口服氢氧化铝后有显著改善。