Two cases of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis.

Ahn, Jeong Cheon; Joh, Weon Yong; In, Kwang Ho; Kang, Kyung Ho; Yoo, Se Hwa

doi:1992.39.6.542

Tuberc Respir Dis > Volume 39(6); 1992 > Article

Case Report

Tuberculosis and Respiratory Diseases 1992;39(6):542-547.
DOI: https://doi.org/10.4046/trd.1992.39.6.542 Published online December 1, 1992.

Two cases of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis.

Jeong Cheon Ahn, Weon Yong Joh, Kwang Ho In, Kyung Ho Kang, Se Hwa Yoo

Department of lnterna! Medicine, College of Medicine, Korea University, Seou!, Korea

Abstract

Tuberous sclerosis is an autosomal dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceum. Associated lesions include retinal phacomata, shagreen patches, subungual 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 literature.

Key Words: Tuberous sclerosis, Pulmonary Lymphangioleiomyomatosis