A Case of Pulmonary Lymphangioleiomyomatosis Associated with Tuberous Sclerosis and Renal Angiomyolipoma

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)