PERIPHERAL AND CORONARY-ARTERY DISSECTION S IN A YOUNG WOMAN - A RARECASE OF TYPE-IV EHLERS-DANLOS SYNDROME

Spontaneous coronary dissection is rare and the diagnosis is usually p ost-mortem. Less than 60 cases have been diagnosed at coronary angiogr aphy. The authors report, to the best of their knowledge, the first ca se of multiple spontaneous coronary artery dissections in a type IV Eh lers-Danlos syndrome in a young woman admitted to hospital for acute m yocardial infarction. She had a previous history of regressive complet e tetraplegia due to dissection of the basilar artery and episodes of dizziness related to a dissecting aneurysm of the left vertebral arter y. The diagnosis of type IV Ehlers-Danlos syndrome was established aft er skin biopsy had shown typical histological changes. The patient die d several months later after an acute abdominal syndrome probably rela ted to dissection of the aorta. An autopsy was refused by her family. The authors believe this to be the first case of spontaneous coronary dissection related to a type IV Ehlers-Danlos syndrome.