Tatlıpınar Sinan, Şahan Berna, Altunsoy Muhsin
Yeditepe University Faculty of Medicine, Department of Ophthalmology, İstanbul, Turkey.
Turk J Ophthalmol. 2015 Dec;45(6):268-270. doi: 10.4274/tjo.84756. Epub 2015 Dec 5.
Pseudoxanthoma elasticum (PXE) is a genetic multisystemic disorder affecting the skin, eyes and cardiovascular system. Basic fundoscopic findings in PXE result from Bruch's membrane involvement. The most important fundoscopic findings are angioid streaks. Other significant ocular findings are peau d'orange appearance, optic disc drusen, pattern dystrophy-like macular appearance, comet lesions, and choroidal neovascularization. Comet lesions are a pathognomonic ocular finding for PXE. The presence of both angioid streaks in the fundus and typical skin lesions should alert clinicians to PXE. Herein, we present two PXE cases with comet lesions.
弹性假黄瘤(PXE)是一种影响皮肤、眼睛和心血管系统的遗传性多系统疾病。PXE的基本眼底检查结果是由于布鲁赫膜受累所致。最重要的眼底检查结果是血管样条纹。其他重要的眼部表现包括橘皮样外观、视盘玻璃膜疣、图案营养不良样黄斑外观、彗星样病变和脉络膜新生血管。彗星样病变是PXE的特征性眼部表现。眼底出现血管样条纹和典型的皮肤病变应提醒临床医生注意PXE。在此,我们报告两例伴有彗星样病变的PXE病例。