저자들은 신장의 혈관근 지방종과 간질로 결절성 경화증을 진단 받고 추적관찰 중인 환자에서 발생한 점진적인 호흡곤란으로 시행한 흉부 전산화 단층 촬영과 병리 조직 소견에서 HMB-45 양성의 폐 림프관 혈관종증을 진단한 1예를 경험하였기에 문헌과 함께 보고하는 바이다.

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. (Korean J Med 2011;81:775-779)