Progressive Pulmonary Emphysema Associated with Congenital Generalized Elastolysis (Cutis Laxa)

Abstract

A case of progressive pulmonary emphysema, segmental pulmonary artery stenosis, and cutis laxa is reported. The generalized disorder of connective tissue appeared to be responsible for the multisystemic abnormalities. The pulmonary emphysema in generalized elastolysis is postulated to be the result of diffuse defects in the elastic tissue involving the entire respiratory tree, producing obstruction similar to that seen in adult emphysema.