Abstract
Abstract Hemopneumothorax is most commonly encountered following trauma. Spontaneous hemopneumothorax is rare. Causes of spontaneous hemopneumothorax include congenital cystic lung diseases, bleeding disorders, collagen vascular diseases such as rheumatoid arthritis, systemic lupus erythematosus, and complications of lung malignancies. Vascular Ehlers–Danlos syndrome (vEDS) is a life-threatening rare genetic disorder with a high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. This report is of a young male presenting with a history of recurrent hemopneumothorax. He was diagnosed with vEDS by identifying a COL3A1 gene mutation. This report emphasizes the importance of genetic studies when young patients present with unexplained and recurrent hemopneumothorax. Genetic counseling and close monitoring are imperative for the prevention of severe complications.
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