Pulmonary manifestations and management of proteus syndrome.

[proteus syndrome]

Proteus syndrome is a very rare , sporadic and congenital condition that is characterized by postnatal mosaic overgrowth . This disorder is thought to be caused by a somatic gene mutation , but the exact etiology is unknown . Commonly involved tissues include connective tissue , bone , skin and the central nervous system . Another less common symptom involves pulmonary emphysematous changes . This report documents a 25 -year -old man with Proteus syndrome who presented with progressive exertional dyspnea and asymmetric overgrowth of his extremities . He underwent left pneumonectomy and his postoperative course was uneventful . Lung tissue showed emphysematous changes with multiple bulla formation and scattered calcification . We also review recent literature related to pulmonary manifestations and management of Proteus syndrome .