CONGENITAL CUTIS LAXA - A REPORT OF 2 CAS ES WITH A REVIEW OF THE LITERATURE

Congenital cutis laxa is a rare heritable disorder characterized by ab normally loose skin with a reduction in elastic tissue throughout the dermis. It occurs in two forms, a severe autosomal recessive form asso ciated with visceral abnormalities (including pulmonary emphysema), an d a dominant autosomal form with abnormalities confined to the skin an d an essentially benign prognosis. Two cases of cutis laxa in genetica lly-related individuals are reported. The family history and the clini cal picture characterized by severe manifestations with early pulmonar y 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 subsequ ent children.