Hemoptysis in Proteus syndrome

We report a patient with Proteus syndrome who presented with massive hemoptysis. Proteus syndrome is a rare congenital disorder characterized by overgrowth of tissues, including skin, vasculature, and skeleton. The asymmetric overgrowth of these tissues result in marked deformity and subsequent medical complications known to include deep venous thrombosis and subsequent pulmonary embolism. Although vascular malformations of visceral organs have been previously reported in Proteus syndrome, endobronchial involvement resulting in massive hemoptysis, as reported here, represents a novel finding. This complication is the result of bronchial neovascularization caused by Proteus syndrome and not pulmonary embolism. We discuss these findings and review the literature regarding pulmonary complications of Proteus syndrome.