Systemic vasculitis presents a group of clinically pathologic syndromes with acute inflammatory process in its basis which commonly occurs on small blood vessels destroying their walls with fibrinoid necrosis. It often occurs on several organs and systems depending on the form, size and number of the diseased blood vessels. This paper is a case report on a female patient suffering from a serious systemic necrotizing vasculitis with clinically demonstrated ulceronecrotizing skin changes and purpura together with appearance of coronary ischemia; rhythmic disorders of heart rate and gastrointestinal bleeding and cardiac arrest in one moment. It contains a description of the course of the disease and a good result after application of immunosuppressive therapy with high doses of corticosteroids, cyclosporin A and plasmaphereses.