Generalized elastolysis (cutis laxa).

ABSTRACT

Generalized elastolysis is a rare and unique systemic disorder of connective tissue in which the elastic fibers suddenly become fragmented, disorganized and fewer in number with the resultant production of such entities as cutis laxa, emphysema, aortic aneurysms and bowel diverticula appearing in the organ system involved, that is, skin, lung, vasculature or gastrointestinal tract. Presented here are three cases that, illustrate the typical features of this condition. In addition, 14 more cases were retrieved from the literature and the information reviewed. Various etiologic factors relating to the synthesis and degradation of elastic tissue are discussed in light of recent findings in the biochemistry of connective tissue.