Lymphangioleiomyomatosis Presenting as Recurrent Pneumothorax

Lymphangioleiomyomatosis (LAM) is a disorder that causes cystic disease in the lungs. This condition is associated with renal angiomyolipomas and commonly occurs in individuals with tuberous sclerosis. Despite its frequent association with tuberous sclerosis, LAM is a rare condition and is often underdiagnosed. An identification of the array of signs for LAM is necessary to start the patient on appropriate long-term guideline-directed medical therapy. A 24-year-old female patient with a past medical history of tuberous sclerosis, gestational hypertension, stable renal angiomyolipoma, and recent pneumothorax presented to our emergency department complaining of four weeks of productive cough. On presentation, she was found to have stable vital signs, and on examination, she had absent breath sounds in the right basilar and anterior mid-lung field compared to the left. Subsequent imaging confirmed a recurrence of pneumothorax from her visit three months prior to her current presentation as well as progressive cystic lung disease and an unchanged angiomyolipoma, suggestive of LAM. We present this article with the hope of raising the index of suspicion for LAM in the setting of the particular signs and symptoms and to encourage prompt stabilization of the patient and initiation of guideline-directed medical therapy and strict follow-up to provide the greatest possible improvement in the patient’s quality of life.