Sayers C P, Goltz R W, Mottiaz J
J Invest Dermatol. 1975 Nov;65(5):451-7. doi: 10.1111/1523-1747.ep12608193.
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 patient's 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 cutix 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.
我们研究了马凡氏综合征患者、全身性弹性组织溶解症患者、其他原因引起的肺气肿患者以及死于无关病因者肺部的弹性组织变化。在马凡氏综合征患者的弹性组织纤维中可见退行性变化，程度从轻度到重度不等。皮肤松弛症患者肺部的弹性纤维在光镜和电镜下显示出与先前在皮肤和血管中报道的相同形态学变化。虽然肺泡间隔中单个弹性纤维的改变看起来相似，但在皮肤松弛症中几乎所有纤维都严重受累，而在马凡氏综合征中受累程度没那么严重，也并非影响所有纤维。在检查皮肤松弛症或马凡氏综合征患者的肺切片时，两者都很容易与正常和异常对照区分开来。
