Acquired cutis laxa. Primary generalized elastolysis

Abstract

Acquired cutis laxa is part of a generalized elastolysis with involvement of the skin and such organs as the lungs, aorta, and gastrointestinal tract. One patient with acquired cutis laxa died after 17 years of progressive disease leading to severe laxity of the skin, pulmonary emphysema, hiatus hernia, Zenker's bilobed diverticulum, inguinal hernia, prolapse of the gastric mucosa and gallbladder, and diverticulosis of the large bowel. The second patient 9 years of age, following a generalized vesicular eruption, developed cutis laxa of the face which has progressed despite plastic surgical repair. Acquired cutis laxa may be accompanied by skin manifestations such as erythema multiforme, urticaria, or a nonspecific dermatitis. Elastic fibers are diminished throughout the dermis with fragmentation and granularity. Elastic fibers throughout the body (the aorta and the lungs, in particular) show these same changes.