Brazzelli V, Dell'Orbo C, Borroni G, Bollati A, Montecucco C, Cerimele D, Rabbiosi G
Department of Human and Hereditary Pathology, University of Cagliari, Italy.
Am J Dermatopathol. 1992 Feb;14(1):42-9. doi: 10.1097/00000372-199202000-00007.
We performed an electron-microscopic study on calcium deposits in two patients with calcifying scleroderma (CRST syndrome). Calcium deposits were detected both intracellularly in the mitochondria of phagocytic cells and extracellularly. Needle-like crystals measuring up to 4,500 A in length and approximately 60 A in width were present in both the Von Kossa-positive regions and the apparently normal dermal areas around the calcification sites. Although the fibrillar matrix's ultrastructure was normal, hollow oxytalan fibrils were detected. Slightly electron-opaque, star-shaped material was observed among the fibrillar component of the matrix (matrix granules), which is the ultrastructural expression of some types of proteoglycans containing keratan sulphate and chondroitin sulphate. These granules cannot be detected in normal dermis. The extrafibrillary calcium deposits on these mucopolysaccharide structures may represent an early event in the complex pathogenesis of calcification in the CRST syndrome.
我们对两名患有钙化性硬皮病（CRST综合征）的患者的钙沉积进行了电子显微镜研究。在吞噬细胞的线粒体内和细胞外均检测到钙沉积。在Von Kossa染色阳性区域以及钙化部位周围看似正常的真皮区域中均存在长度达4500埃、宽度约60埃的针状晶体。尽管纤维状基质的超微结构正常，但检测到了中空的弹力纤维。在基质的纤维成分（基质颗粒）中观察到了略带电子不透明的星形物质，这是某些含有硫酸角质素和硫酸软骨素的蛋白聚糖的超微结构表现。在正常真皮中无法检测到这些颗粒。这些粘多糖结构上的纤维外钙沉积可能代表了CRST综合征钙化复杂发病机制中的早期事件。
