Cutis Laxa

Abstract

The condition known as cutis laxa has been reviewed and two examples occurring in brothers reported. One of these children died from complications of his disease and came to autopsy. Extensive histologic, ultramicroscopic, histochemical, and biochemical studies on skin and other organs demonstrated a defect of elastic fibers throughout the body. Review of the literature indicates similar widespread involvement in other cases of this disease. Therefore, the title generalized elastolysis is proposed to express, better than does cutis laxa, the extensive distribution in connective tissues of this rare but serious disease. The occurrence of this syndrome in siblings and the evidence of parental consanguinity in several pedigrees strongly suggest an autosomal recessive inheritance.