Cutis Laxa (Generalized Elastolysis) A Report of Four Cases with Autopsy Findings

Abstract

Children (4) with cutis laxa (generalized elastolysis) are reported. The first 3 cases were siblings from a Canadian Indian Family and the 4th case was the only affected child in an American Black family. Loose and sagging skin folded over the face, neck and trunk, gave a premature senile appearance. Post-mortem examination was performed on the first 3 cases. The most common and serious visceral involvement was development of pulmonary emphysema. This was present in 2 autopsied cases and was demonstrated by chest X-ray in the 4th case. Other abnormalities included large inguinal and perineal hernia, rectal diverticulum and multiple diverticulae of the urinary bladder.