Journal of Pulmonary Medicine.

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A Rare Case of Sporadic Lymphangioleiomyomatosis with Manifestation of Recurrent Pneumothorax in a Premenopausal Female

Sporadic lymphangioleiomyomatosis (LAM) is a rare multisystem disorder affecting approximately 3 to 8 per million women. Our patient, a 42-year-old nulligravida Hispanic female with recurrent pneumothoraces presented to the Emergency Department (ED) with a chief complaint of shortness of breath and exertional chest tightness that progressively worsened over four days. Upon arrival to the ED she was tachypneic and hypoxic, with chest x-ray revealing large bilateral pneumothoraces and chest computed tomography (CT) from a previous encounter showed dozens of air-filled cavities in both lungs. Already possessing the diagnosis of LAM upon presentation and having had her most recent pneumothorax 3 months prior, our patient reported to the hospital once she recognized her symptoms as another relapse. Cavitated cystic blebs within the lung parenchyma are believed to be the reason behind recurrent pneumothoraces in those with LAM, such as with our patient. The importance of educating patients with rare conditions is illustrated in this case. Our patient was aware of the importance of going to an ED upon onset of dyspnea due to her condition, and was therefore able to receive appropriate care.

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