Miller M, Carton F X
Ann Dermatol Venereol. 1980 Aug-Sep;107(8-9):841-5.
Vascular lesions have been discovered by a number of authors in children's dermatomyositis, when they are not ordinary found in adults. Affecting capillaries, arterioles and veinlets, vasculitis appears as an hyperplasia of intima, then as an endothelial necrosis and formation of thrombi. Usually seen with light microscope, these lesions are sometimes only revealed by electron microscope. These vascular lesions are observed in the muscle and mainly in the perimysial interstitial tissue, in the skin, and in the various visceral localisations of the disease: pulmonary, ocular, renal, nervous and mainly digestives. The ulcerations of the digestive tube are due to little infarcts secondary to submucous arteriolar occlusions. The presence of necrotizing microangiopathy in the muscle, in the skin and in the viscera, and the observation by an author of macroglobulin, gammaglobulin and C3 deposits in the vascular walls allows to issue an aetiopathogenic hypothesis. The vascular lesions could be due, at least partly, to immune complex deposits, explained by an allergic mechanism of type III of Gell and Coombs. This process could be released by a viral factor which has to be determined. This pathogeny could bring children's dermatomyositis nearer to the other circulating immune complexe collagenosis: S. L. E., periarteritis nodosa with which many transition form exist, and could separate it to the adults dermatomyositis, which would rather be a disease due to an exacerbation of the delayed hypersensitivity type IV of Gell and Coombs.
许多作者在儿童皮肌炎中发现了血管病变,而在成人中通常不会出现这种情况。血管炎累及毛细血管、小动脉和小静脉,表现为内膜增生,随后出现内皮坏死和血栓形成。这些病变通常在光学显微镜下可见,有时仅通过电子显微镜才能发现。这些血管病变见于肌肉,主要在肌束膜间质组织中,也见于皮肤以及该疾病的各种内脏部位:肺部、眼部、肾脏、神经,主要是消化系统。消化道溃疡是由于黏膜下小动脉闭塞继发的小梗死所致。肌肉、皮肤和内脏中存在坏死性微血管病,以及一位作者观察到血管壁中有巨球蛋白、γ球蛋白和C3沉积,这使得我们可以提出一种病因学假说。血管病变可能至少部分是由于免疫复合物沉积,这可以用盖尔和库姆斯的III型过敏机制来解释。这个过程可能由一种有待确定的病毒因素引发。这种发病机制可能使儿童皮肌炎更接近于其他循环免疫复合物性胶原病:系统性红斑狼疮、结节性多动脉炎,它们之间存在许多过渡形式,并且可以将其与成人皮肌炎区分开来,成人皮肌炎更可能是一种由盖尔和库姆斯的IV型迟发型超敏反应加剧引起的疾病。