Pulmonary lymphangioleiomyomatosis masquerading as unilateral heterogeneous emphysema

Pulmonary lymphangioleiomyomatosis (LAM) is a rare, well-described pathology and usually is exclusive to females of a reproductive age. We present a 45 year-old lady who presented to the surgeons 1 year after an admission with acute dyspnoea following influenza infection. Initial computed tomography imaging findings demonstrated severe, heterogenous right-sided bullous emphysematous changes, but histopathological analysis of the post-operative specimen favoured a diagnosis of LAM. This case demonstrates the importance of considering LAM as a differential diagnosis for findings of unilateral emphysema or lobar emphysema, in which alpha 1-antitrypsin deficiency has been excluded and in those without a significant smoking history.