[A little known cause of chronic renal insufficiency in the adult: systemic tuberous sclerosis].

Background: Angiomyolipomas and renal cysts are commonly observed in patients with systemic tuberous sclerosis. Hemorrhagic complications related to the angiomyolipomas are well known.

Case Report: A 63-year old woman was hospitalized for severe chronic renal failure. Physical examination revealed skin manifestations of systemic tuberous sclerosis, which the patient had hidden to date. The radiographic work-up demonstrated complete disorganization of the renal parenchyma in both kidneys due to multiple cysts and angiomyolipomas.

Discussion: Chronic renal failure can be the inaugural manifestation in low-grade cases of systemic tuberous sclerosis which may go undiagnosed for years allowing progressive destruction of the kidneys.