Journal of Investigative Dermatology (1975) 65, 451–457; doi:10.1111/1523-1747.ep12608193
PULMONARY ELASTIC TISSUE IN GENERALIZED ELASTOLYSIS (CUTIS LAXA) AND MARFAN'S SYNDROME. A LIGHT AND ELECTRON MICROSCOPIC STUDY
C Paul Sayers, Robert W Goltz and Jess Mottaz
Department of Dermatology, University of Minnesota Hospitals, Minneapolis, Minnesota
Top of pageAbstract
We have studied elastic tissue changes in the lungs from patients with Marfan's syndrome, generalized elastolysis, patients with emphysema of other cause, and those dying from unrelated causes. Degenerative changes were seen in the elastic tissue fibers in patients with Marfan's syndrome; they varied from mild to severe. Elastic fibers in the lungs of patients with cutis laxa showed by light and electron microscopy the same morphologic changes previously reported in the skin and vessels. While the alterations in individual elastic fibers in the alveolar septa appeared similar, in cutis laxa there was severe involvement of almost all fibers, whereas in Marfan's syndrome the involvement was not so severe, nor did it affect all fibers. On examination of lung sections from patients with cutis laxa or Marfan's syndrome, both were readily separable from normal and abnormal controls.
Top of pageReferences
- McKusick, VA: The Marian syndrome. Heritable Disorders of Connective Tissue 1972 St Louis, Mosby,
- Reye, RDK, Bale, PM: Elastic tissue in pulmonary emphysema in Marfan's syndrome. Arch Pathol 1973 96: 427–431, | PubMed |
- Goltz, RW, Huit, AM: Cutis laxa. Arch Dermatol 1965 92: 373–387, | Article | PubMed | ChemPort |
- Takebayashi, S, Kubota, I, Takagi, A: Ultrastructural and histochemical studies of vascular lesions in Marfan's syndrome, with report of 4 autopsy cases. Acta Pathol Jap 1973 23: 847–866,
- Hashimoto, K, Kansaki, T: Ultrastructural study of cutis laxa. Arch Dermatol 1975 (in press)
- Gore, I: The pathogenesis of dissecting aneurysm of the aorta. Arch Pathol 1952 53: 142,
- Roark, JW: The Marfan syndrome—report of one case with autopsy, special histological study, and review of the literature. Arch Int Med 1959 103: 123–132,
- Bolande, RP, Tucker, AS: Pulmonary emphysema and other cordiorespiratory lesions as part of the Marfan abiotrophy. Pediatrics 1964 33: 356–366, | PubMed |
- Pinkus, H, Keech, MK, Mehregan, AH: Histopatholopy of striae distensae, with special reference to striae and wound healing in the Marfan syndrome. J Invest Dermatol 1966 46: 283–292, | PubMed | ISI | ChemPort |
