[Rupture of the major artery in incomplete desmogenesis]

A pathomorphological examination of a congenital disease - incomplete desmogenesis (ID) - was made in 5 women aged 19-34 years. They had spontaneous ruptures of the major arteries (subclavial, brachial, splenic, gastric). Microscopically, the wall of the ruptured artery contained no or minimal collagenic structures which looked homogeously, like a mixomatous edema. Content of acid mucopolysaccharides and hyperelastoses was elevated. The rupture was provoked by infections, additional loads, such as pregnancy, etc. Vascular surgery in ID is difficult as arteries often break at suturing while vascular walls are amputated at ligation. In one patient tamponade stopped hemorrhage and saved her.