Shishkina L V, Lazarev V A, Meshcheriakova A V, Printseva O Iu
Arkh Patol. 1993;55(4):16-20.
Results of morphological examination of the skin and vessels in patients with large and giant brain artery aneurysms are presented. Ehlers-Danlos syndrome (type IV) was diagnosed in half of the cases; very thin skin and vessel walls, the disturbance of the collagen fibril structure in dermis, increased permeability and the tendency to rupture of the inner elastic membrane of brain arteries combined with a severe intimal atrophy. The absence of the III type collagen in the skin biopsies was shown immunohistochemically. The feasibility of the morphological diagnosis of this syndrome by skin biopsies and the significance of such diagnosis for prevention of complications in a surgical treatment are demonstrated.
本文展示了大脑大型和巨型动脉瘤患者皮肤和血管的形态学检查结果。半数病例被诊断为埃勒斯-当洛综合征（IV型）；皮肤和血管壁极薄，真皮中胶原纤维结构紊乱，通透性增加，脑动脉内弹性膜有破裂倾向并伴有严重内膜萎缩。免疫组织化学显示皮肤活检中缺乏III型胶原蛋白。证明了通过皮肤活检对该综合征进行形态学诊断的可行性以及这种诊断对预防手术治疗并发症的意义。
