A 45-year-old man who presented with acute renal insufficiency is described. He deteriorated during haemodialysis, developed severe gastrointestinal bleedings, and died two months after his initial admission. Histopathological changes responsible for his renal disease were focal narrowing and occlusion of the arteries of the kidneys due to concentric intimal proliferation. These findings are consistent with those described in Köhlmeier-Degos’ disease, a fatal cutaneo-intestinal arterio-occlusive syndrome. Therefore we consider our patient to represent a case of this disease, which may be added to the list of rare causes of acute renal failure.
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