[Chronic renal failure in bourneville-pringle's disease (author's transl)]

Abstract

In a 38-year-old woman the process of developing chronic renal failure in Bournevill-Pringle's disease is described. The diagnosis is based on the typical skin lesions as well as the familiary affliction concerning four generations. Only in the fourth generation a brain involvement is detectable. In the presented case cystic mixed tumors of the kidneys are responsible for the renal insufficiency, combined with consecutive pyelonephritis. According to the changing clinical expressivity of this congenital disease it can be deduced, that Pringle's disease is in this case an abortive form of tuberous sclerosis. The basic lesion of tuberous sclerosis is hamartomatous tissue change. The brain, skin, bones and kidneys are the most commonly affected sites.