Department of Ophthalmology, University Hospital, Linköping, Sweden.
Acta Ophthalmol. 2010 Jun;88(4):394-400. doi: 10.1111/j.1755-3768.2009.01548.x. Epub 2009 Aug 14.
The aim of this study was to describe morphological changes in Dystrophia Smolandiensis, a corneal disease that is characterized by recurrent corneal erosive episodes and the formation of central corneal keloid-like opacities in approximately half of those affected.
The corneas of seven affected individuals were examined using in-vivo confocal microscopy. Specimens of one primary corneal graft, one regraft and one biopsied keloid-like region--all obtained from members of a large family with the disease--were re-examined with a light microscope. Sections were stained with Congo red and analysed immunohistochemically for fibronectin and S100A4.
Light microscopic examination revealed epithelial hyperplasia, absence of Bowman's layer and subepithelial fibrosis. Fibronectin was expressed in the area of subepithelial fibrosis, and the keratocytes in this area generally expressed S100A4. The biopsy specimen stained positive for Congo red, suggesting an amyloid deposit. In-vivo confocal microscopy confirmed epithelial abnormalities, loss of Bowman's layer and significant alterations of the subbasal nerve plexus in affected individuals.
The morphological picture in Dystrophia Smolandiensis is novel for a condition dominated by recurrent corneal erosions at the clinical level. Although no single morphological feature unique to the disease could be found, the general morphological pattern of pathology (true keloid formation, absence of Bowman's layer, subepithelial fibrosis and abnormal subbasal nerves) probably reflects a novel phenotypic expression of the healing response to recurrent erosion of the corneal epithelium. However, the pathogenesis of Dystrophia Smolandiensis remains to be elucidated fully.
本研究旨在描述 Smolandiensis 营养不良症的形态学变化。该疾病的特征为反复性角膜侵蚀性病变，并在约半数患者中形成中央角膜类瘢痕样混浊。
使用活体共聚焦显微镜检查 7 名受影响个体的角膜。取自患有该疾病的一个大家族的成员的一个原发性角膜移植物、一个再移植和一个活检的类瘢痕样区域的标本，用光学显微镜进行重新检查。对切片进行刚果红染色，并进行纤维连接蛋白和 S100A4 的免疫组织化学分析。
光学显微镜检查显示上皮细胞增生、Bowman 层缺失和上皮下纤维化。纤维连接蛋白在亚上皮纤维化区域表达，该区域的角朊细胞通常表达 S100A4。活检标本对刚果红染色呈阳性，提示有淀粉样物质沉积。活体共聚焦显微镜证实受影响个体的上皮异常、Bowman 层缺失和基底下神经丛的显著改变。
在以临床水平反复性角膜侵蚀为主的疾病中，Smolandiensis 营养不良症的形态学表现是新颖的。尽管未发现疾病所特有的单一形态学特征，但病理的一般形态模式（真正的瘢痕形成、Bowman 层缺失、上皮下纤维化和异常的基底下神经）可能反映了对角膜上皮反复侵蚀的愈合反应的一种新型表型表达。然而，Smolandiensis 营养不良症的发病机制仍有待充分阐明。
