Nika Melisa, Besirli Cagri G
Department of Ophthalmology and Visual Sciences, University of Michigan Medical School, Ann Arbor, Michigan, Kellogg Eye Center, 1000 Wall Street, Ann Arbor, MI 48105, USA.
Int J Ophthalmol. 2011;4(4):449-51. doi: 10.3980/j.issn.2222-3959.2011.04.25. Epub 2011 Aug 18.
To report a case of pseudoxanthoma elasticum (PXE) in a 48 year old woman that presented with bilateral blurry vision.
A case report
A 48-year-old woman presented with bilateral blurry vision and right eye metamorphopsia. The patient had a history of angioid streaks in the left eye ten years ago for which she had received laser surgery and had poor residual vision. Visual acuity was 20/60 in the right eye and count fingers at 6 feet in the left. Fundus examination showed subretinal hemorrhage and macular thickening on the right and a disciform macular scar with focal atrophic pigment epithelial lesions on the left. Both eyes had angioid streaks and peau d'orange pigmentary pattern of the retina. External examination showed several, yellow skin papules and plaques on the lateral and posterior neck, as well as prominent mental creases. Pathologic examination of skin biopsy confirmed the diagnosis of PXE, showing calcium deposition and fragmented, clumped elastic fibers in the deep reticular dermis. She responded well to intravitreal bevacizumab injections and visual acuity improved to 20/25 OD. Preventative care was emphasized and the patient was referred to cardiology, gastroenterology and human genetics for counseling.
PXE is a multisystem disorder affecting the dermatologic, ocular, and cardiovascular systems. Ophthalmic findings of angioid streaks and choroidal neovascularization in the presence of stereotypical skin changes and prominent mental creases should prompt evaluation for PXE.
报告一例48岁患有双侧视力模糊的弹性假黄瘤（PXE）女性病例。
病例报告
一名48岁女性出现双侧视力模糊及右眼视物变形。该患者10年前左眼有血管样条纹病史，曾接受激光手术，视力残留较差。右眼视力为20/60，左眼在6英尺处只能数手指。眼底检查显示右眼视网膜下出血和黄斑增厚，左眼为盘状黄斑瘢痕伴局灶性萎缩性色素上皮病变。双眼均有血管样条纹及视网膜的橘皮样色素沉着模式。外部检查显示颈部外侧和后部有几个黄色皮肤丘疹和斑块，以及明显的额纹。皮肤活检的病理检查确诊为PXE，显示深层网状真皮中有钙沉积以及弹性纤维断裂和聚集。她对玻璃体内注射贝伐单抗反应良好，右眼视力提高到20/25。强调了预防保健，并将患者转诊至心脏病学、胃肠病学和人类遗传学部门进行咨询。
PXE是一种影响皮肤、眼部和心血管系统的多系统疾病。在存在典型皮肤改变和明显额纹的情况下，出现血管样条纹和脉络膜新生血管的眼科表现应促使对PXE进行评估。
