Vascular involvement in Behçet's disease was first mentioned by Adamantiades and occurs in addition to the classic symptom triad. In the present work an unusually fulminant course of Adamantiades-Behçet disease with a lethal outcome is reported in a 43-year-old male patient of German origin. The first symptoms were painful oedemas of the joints, followed 1 year later by bipolar aphthous lesions and by multiple progressive ulcers of the skin and the oral mucosa. Histologically, pustule formation with underlying vasculitis was diagnosed. In spite of systemic treatment with azathioprine and high-dosed prednisolone, the course was progressive with multiple necrotizing ulcers, and the patient died 18 months after the onset of the disease with signs of heart insufficiency and cachexia. The autopsy findings revealed multiple lesions of ulcerating systemic vasculitis of the skin and other visceral organs, including the lungs and the central nervous system, and also endocarditis verrucosa ulcerosa of the mitral valve.