[Multiple vascular lesions in a patient with Ehlers-Danlos type IV]

Ehlers-Danlos syndrome derives from some defect collagen synthesis or structure. It collects several disorders, clinically and genetically different. The young woman history with silent disease until lethal spontaneous arterial rupture is presented. Post-mortem examination shows type IV syndrome aspects: arterial fragility and right vertebral aneurisma rupture into pleural cavity with consequent fatal emothorax. Moreover there are mitral valve myxomatous degeneration, ascending aorta dilatation, lungs emphysema and meningioma.