A Case of Pulmonary Lymphangioleiomyomatosis Associated with Tuberous Sclerosis and Renal Angiomyolipoma.
- Author:
So Hee PARK
1
;
Ju Woong SON
;
Choul Ki PARK
;
Myung Jae PARK
;
Jee Hong YOO
;
Hong Mo KANG
Author Information
1. Department of Internal Medicine and Pulmonology and Critical Care, Kyung Hee University School of Medicine, Seoul, Korea. faab01@naver.com
- Publication Type:Case Report
- Keywords:
Tuberous sclerosis;
Lymphangioleiomyomatosis;
Renal angiomyolipoma
- MeSH:
Adult;
Angiomyolipoma;
Epilepsy;
Female;
Humans;
Intellectual Disability;
Lymphangioleiomyomatosis;
Myocytes, Smooth Muscle;
Skin;
Tuberous Sclerosis
- From:Korean Journal of Medicine
2011;81(6):775-779
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Tuberous sclerosis is an autosomal dominant disorder characterized by facial skin lesions, epilepsy, and mental retardation. Pulmonary involvement in tuberous sclerosis is rare and shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is characterized by the progressive proliferation of smooth muscle cells and occurs in 0.1-1% of patients with tuberous sclerosis. We encountered a case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis and bilateral renal angiomyolipoma in a 31-year-old female patient. This case is reported here along with a brief review of the literature.