Literature DB >> 707540

Generalized elastolysis (cutis laxa).

R B Harris, M R Heaphy, H O Perry.   

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.

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Year:  1978        PMID: 707540     DOI: 10.1016/0002-9343(78)90801-x

Source DB:  PubMed          Journal:  Am J Med        ISSN: 0002-9343            Impact factor:   4.965


  4 in total

Review 1.  Genetics of the extracellular matrix in aortic aneurysmal diseases.

Authors:  Chien-Jung Lin; Chieh-Yu Lin; Nathan O Stitziel
Journal:  Matrix Biol       Date:  2018-04-12       Impact factor: 11.583

Review 2.  alpha-1-Antitrypsin and the pathogenesis of emphysema.

Authors:  R A Stockley
Journal:  Lung       Date:  1987       Impact factor: 2.584

3.  Pulmonary disease in patients with Marfan syndrome.

Authors:  J R Wood; D Bellamy; A H Child; K M Citron
Journal:  Thorax       Date:  1984-10       Impact factor: 9.139

4.  Acquired Localized Cutis Laxa due to Increased Elastin Turnover.

Authors:  Rie Harboe Nygaard; Scott Maynard; Peter Schjerling; Michael Kjaer; Klaus Qvortrup; Vilhelm A Bohr; Lene J Rasmussen; Gregor B E Jemec; Michael Heidenheim
Journal:  Case Rep Dermatol       Date:  2016-02-13
  4 in total

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