Congenital cutis laxa. A report of two cases with a review of the literature.

Congenital cutis laxa is a rare heritable disorder characterized by abnormally loose skin with a reduction in elastic tissue throughout the dermis. It occurs in two forms, a severe autosomal recessive form associated with visceral abnormalities (including pulmonary emphysema), and a dominant autosomal form with abnormalities confined to the skin and an essentially benign prognosis. Two cases of cutis laxa in genetically-related individuals are reported. The family history and the clinical picture characterized by severe manifestations with early pulmonary complications were consistent with the autosomal recessive form, The two forms of cutis laxa should be differentiated during the neonatal period to allow evaluation of the prognosis and of the risk to subsequent children.