腎病変が著明であったBourneville-Pringle母斑症の1例

Abstract

A 33-year-old man was first seen with flank pain on the left side and gross hematuria. Past history showed autograft of the skin from the chest wall to the face because of facial sebaceous adenomas. Physical examination revealed a mass palpable at the hypochondrium on the left. Facial sebaceous adenomas were seen symmetrically distributed around the nasolabial folds. X-ray examination of the head showed multiple calcified lesions. Biopsy of sebaceous adenoma disclosed histological finding compatible with Bourneville-Pringle's phakomatosis. Excretory urography and renal arteriography suggested renal tumor associated with Bourneville-Pringle's phakomatosis. Biopsy of the kidney disclosed angiomyolipoma.