"Aberrant elastic" in elastofibroma: an immunohistochemical and ultrastructural study.
"Aberrant elastic" in elastofibroma: an immunohistochemical and ultrastructural study.
复制标题
弹力纤维瘤中的“异常弹性”：一项免疫组织化学和超微结构研究。
作者：
H. Kahn;W. Hanna
弹力纤维瘤是一种罕见的病变，其特征是存在大量异常的弹力纤维，具有独特的形态、成纤维细胞的增殖和胶原沉积。弹性纤维的形态改变是一种退行性现象，还是由于异常的弹力发生引起的，目前仍存在争议。我们用溶菌酶抗体对胎儿皮肤和3例弹性纤维瘤进行了光镜和免疫组织化学研究，对1例弹性纤维瘤进行了电子显微镜(EM)观察。我们以前的研究表明，成人皮肤的正常弹性纤维不显示溶菌酶，而日光弹力增生症和弹性假性黄瘤的异常弹性纤维对溶菌酶呈阳性反应。在胎儿皮肤和所有三例弹性纤维瘤中，弹性纤维均为溶菌酶阴性。透射电子显微镜显示弹力纤维呈花状结构，由正常或退化的弹性蛋白组成的中心核心，周围环绕着电子密度不同的颗粒状和丝状物质的辐射辐条，表明微纤维的结构和组织结构异常。异常弹力中没有溶菌酶并不能区分是胎儿弹力还是成人弹力产生过多。然而，在超微结构水平上看到的过多的微纤维表明，可能存在过多的胎儿弹性生产。弹力纤维与成纤维细胞关系密切，常出现在成纤维细胞的小窝内，提示异常弹力纤维是由成纤维细胞产生的。我们的研究表明，弹性纤维瘤中异常弹力纤维的产生与随后的退变有关。
Elastofibroma is a rare lesion characterized by the presence of abundant abnormal elastic fibers with a unique morphology, fibroblastic proliferation, and collagen deposition. Whether the altered morphology of the elastic fibers is a degenerative phenomenon or is due to abnormal elastogenesis is controversial. We studied fetal skin and three cases of elastofibroma by light microscopy and immunohistochemistry using an antibody to lysozyme, and one case of elastofibroma by electron microscopy (EM). Our previous studies have shown that normal elastic fibers in adult skin do not stain for lysozyme whereas abnormal elastic fibers in solar elastosis and pseudoxanthoma elasticum react positively for lysozyme. In the fetal skin and all three cases of elastofibroma the elastic fibers were negative for lysozyme. EM showed the abnormal flowerlike configuration of the elastic fibers, which consisted of a central core of normal or degenerating elastin surrounded by radiating spokes of granular and filamentous material of variable electron densities, suggesting that the structure and organization of the microfibrils is abnormal. The absence of lysozyme in the aberrant elastic did not differentiate whether there was excessive production of fetal or adult elastic. However, the excessive amount of microfibrils seen at the ultrastructural level suggests that there may be excessive fetal elastic production. The elastic fibers were intimately related to the fibroblasts and were often present within their caveolae, suggesting that the abnormal elastic fibers are produced by the fibroblast. Our study suggests that abnormal elastogenesis with subsequent degeneration plays a role in the production of the abnormal elastic fibers in elastofibroma.