Kaufman S C, Beuerman R W, Goldberg D
Lions Eye Research Laboratories, LSU Eye Center, Louisiana State University Medical Center School of Medicine, New Orleans 70112-2234, USA.
Metab Pediatr Syst Ophthalmol (1985). 1995;18(1-4):1-4.
We present a case of bilateral, primary, localized, corneal amyloid deposition with a unique appearance not, to our knowledge, previously described. The patient was a 73-year-old white man who complained of glare. Best corrected visual acuity was 20/25 in both eyes. Slit lamp examination revealed subepithelial/anterior stromal white-gray deposits in the peripheral cornea in both eyes. The corneal surface was smooth with no elevated areas. Superficial corneal scrapings showed focal deposits of an amorphous, glass-like material below the epithelial surface and within Bowman's membrane, which stained with Congo red. We examined the amyloid deposits using the confocal microscope to obtain high magnification images in vivo. The confocal microscopic views showed intercellular, cotton candy-like, fibrillar amyloid material scattered throughout the anterior stroma. The use of this instrument may permit clinical diagnosis of this rare disorder without the need for biopsy.
我们报告一例双侧原发性局限性角膜淀粉样变性病例,其外观独特,据我们所知,此前未有描述。患者为一名73岁白人男性,主诉畏光。双眼最佳矫正视力均为20/25。裂隙灯检查发现双眼周边角膜上皮下/前基质层有灰白色沉积物。角膜表面光滑,无隆起区域。角膜表面刮片显示上皮表面下方和Bowman膜内有局灶性无定形玻璃样物质沉积,刚果红染色阳性。我们使用共聚焦显微镜检查淀粉样沉积物,以在体内获得高倍图像。共聚焦显微镜观察显示,细胞间有棉花糖样的纤维状淀粉样物质散在分布于整个前基质层。使用该仪器可在无需活检的情况下对这种罕见疾病进行临床诊断。