All issues > Volume 25(11); 1982

Case Report

J Korean Pediatr Soc. 1982;25(11):1170-1174. Published online November 30, 1982.

A Case of Tuberous Sclelosis with Renal Angiomyolipoma.

H K Park, H J Park, H J Chun, M J Shin, J S Choi

¹Dept, of Pediatrics, Seoul Red Croes Hospital, Seoul, Korea
²Dept, of Pathology, College of Medicine, Korea Univ.，Seoul, Korea

Abstract

Tuberous sclerosis is a heredofamilial disease and is a multisystem disorder affecting primarily tissues derived from ectoderm, such as skin, eye, central and peripheral nervous system, but also involving organs of mesodermal and endodermal origins, such as heart, lungs, kidneys, bones and GI tract. It is characterized by classical triad of mental retard- ation, epilepsy and angiofibroma. We experienced a full blown case of tuberous sclerosis in 14-year old boy. He has the skin lesions such as angiofibroma, shagreen patch, depigmented nevi and flattened fibroma, and sclerotic bone changes, epilepsy, EEG abnormalities, calcified densities on simple X-ray and "brain CT scan, and mental retradation. And also he has right huge renal angiomyolipoma, which was histogically confirmed. Herein, a case of tuberous sclerosis was presented with a brief review of the related literatures.

Keywords :Tuberous sclerosis, Angiofibroma, Shagreen patch, Depigmented nevi, Angiomyolipoma.