A rare case of lymphangioleiomyomatosis with recurrent pneumothorax

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology that traditionally affects young women of childbearing or premenopausal age. It is characterized by proliferation of atypical smooth muscle cells, preferentially along bronchovascular structures that cause progressive respiratory failure. Owing to its unusual and nonspecific presenting symptoms, patients often receive missed or delayed diagnosis. This disease occurs sporadically or in association with the genetic disease-tuberous sclerosis complex. Recurrent pneumothorax is the hallmark of LAM. We present a 16-year-old young female having recurrent pneumothorax with LAM.

Keywords

• Lymphangioleiomyomatosis
• pneumothorax
• tuberous sclerosis