Two Cases of Pulmonary Lymphangioleiomyomatosis Associated with Tuberous Sclerosis

결절성 경화증에 동반된 폐의 임파관평활근종증 2예

Tuberous sclerosis is an autosomal dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceum. Associated lesions include retinal phacomata, shagreen patches, subungal fibromata, and benign visceral tumors such as pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis occurs exclusively in women, usually during the child-bearing years, and is characterized by proliferation of smooth muscle along the lymphatic vessels of the lung, thorax, abdomen. Proliferation of smooth muscle results in interstitial and obstructive lung disease, recurrent pneumothorax, and chylous pleural effusions. We saw two cases of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in women of reproductive age. We report the cases with a brief review of the literatures.

결절성경화증과 동반된 폐임파관평활근종증은 매우 드문 질환으로서 가임기의 여성에게 국한되어 발생한다. 저자들은 결절성경화증이 있던 39세의 여자 환자에게서 반복되는 자연기흉으로 폐조직 생검결과 폐임파관 평활근종증으로 확진된 예와 양측신 혈관근지반종을 동반한 결절성 경화증을 갖은 32세 여자 환자에서 호흡기 증상은 없었으나 흉부 X-선 및 고해상도 흉부 컴퓨터 단층 촬영상 폐임파관 평활근종증을 보였던 예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.