[Skin spots, skin nodules, gangrene of the toes]

A 42-year-old man with multiple cutaneous neurofibromacules, café-au-lait and gangrene of toes was diagnosed having neurofibromatosis in 1976. An aortography performed because of severe hypertension had shown a coarctation and bilateral renal artery stenosis in 1982. Conservative antihypertensive therapy with an ACE-inhibitor, a vasodilatating agent and a diuretic resulted in gradual normalisation of blood pressure values. A recent follow-up revealed minimal progression of neurofibromatosis and hypertensive lesions documenting a benign course for over 13 years.