Am J Med. 1978 Nov;65(5):815-22.

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.

PMID:: 707540; [PubMed - indexed for MEDLINE]

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[Cutis laxa syndrome. Clinical, histologic and ultrastructural study of a new variant]. [Minerva Pediatr. 1989]
[Cutis laxa syndrome. Clinical, histologic and ultrastructural study of a new variant].
Pitzalis G, Bonamico M, Mariani P, Palmieri G, Ceccamea A, Signoretti A. Minerva Pediatr. 1989 Apr; 41(4):193-7.
A case of congenital cutis laxa (generalized elastolysis). [Can Respir J. 2005]
A case of congenital cutis laxa (generalized elastolysis).
Champion P, Ryan F. Can Respir J. 2005 Apr; 12(3):151-2.
Pulmonary elastic tissue in generalized elastolysis (cutis laxa) and Marfan's syndrome: a light and electron microscopic study. [J Invest Dermatol. 1975]
Pulmonary elastic tissue in generalized elastolysis (cutis laxa) and Marfan's syndrome: a light and electron microscopic study.
Sayers CP, Goltz RW, Mottiaz J. J Invest Dermatol. 1975 Nov; 65(5):451-7.
Review [Acquired mid-dermal elastolysis]. [Ann Dermatol Venereol. 1994]
Review [Acquired mid-dermal elastolysis].
Maghraoui S, Grossin M, Crickx B, Blanchet P, Belaïch S. Ann Dermatol Venereol. 1994; 121(3):259-65.
Review Wrinkling due to mid-dermal elastolysis: two cases and literature review. [J Cutan Med Surg. 2000]
Review Wrinkling due to mid-dermal elastolysis: two cases and literature review.
Rao BK, Endzweig CH, Kagen MH, Kriegel D, Freeman RG. J Cutan Med Surg. 2000 Jan; 4(1):40-4.

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Cited by 1 PubMed Central article

Pulmonary disease in patients with Marfan syndrome. [Thorax. 1984]
Pulmonary disease in patients with Marfan syndrome.
Wood JR, Bellamy D, Child AH, Citron KM. Thorax. 1984 Oct; 39(10):780-4.

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