A RARE VARIANT OF GENETICALLY CONFIRMED TUBEROUS SCLEROSIS COMPLEX PRESENTING WITH BILATERAL RENAL ANGIOMYOLIPOMA IN WUNDERLICH SYNDROME

摘要

Introduction: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder with a prevalence of approximately 1 to 6,000- 12,000 worldwide. Renal angiomyolipoma (AML) associated with TSC is often life-threatening due to the greater propensity to develop hemorrhage. A rare condition associated with it is the Wünderlich syndrome (WS), atraumatic renal hemorrhage into the subcapsular and perirenal spaces. We present a case of a patient who presented with the cutaneous and renal features of TSC with bilateral AML in Wünderlich syndrome.