Jornal de doenças pulmonares e tratamento

Pulmonary Emphysema in Cutis Laxa: A Report of Two Cases

Abstract

Cherif H, Dhahri B, Zaibi H, Ben Ammar J and Aouina H

Cutis laxa is a rare connective tissue disorder characterized by progressive looseness of skin, associated with abnormalities of other organs and structures such as lung. Early development of emphysema is usually seen in autosomal recessive forms with death in the early childhood. The autosomal dominant form is usually purely cutaneous and is exceptionally involved in the development of emphysema. In this article, we report two cases of autosomal dominant cutis laxa, both of them had emphysema.