Acquired cutis laxa (Generalized elastolysis): Light and electron microscopic studies

Abstract

The case is described of a 44-yr-old man with acquired cutis laxa. The primary clinical manifestation was an erythema of the chest. Gradually there developed persistent circumscribed lesions in other areas of the skin, spreading restrictive ventilatory insufficiency, elongation of the vocal cord and diverticulosis of the colon were also found, indicating a chronic and widespread disorder. Skin biopsies showed diminution and degeneration of the elastic fibres. The light-microscopic features were subdivided in relation to the various skin manifestations. Ultrastructure of the skin showed various amounts of electron-dense, amorphous material and loosely bound microfibrils in and around elastic and collagen fibres as well as some microfibrils with knobs in the interfibrous and interfibrillar space of the collagen. Most of the collagen fibres, however, were found normal. It is suggested that the first changes in the elastic fibres induced an inflammatory response and mild mucinous changes of the collagen fibres. Furthermore elastic fibres seemed to be replaced by newly formed collagen.