Extensive macular atrophy with pseudodrusen-like appearance (EMAP) was first described in France in 2009 as a symmetric and rapidly progressive form of macular atrophy primarily affecting middle-aged individuals. Despite the recent identification of a significant number of cases in Italy and worldwide, EMAP remains an underrecognized condition. The clinical triad typical of EMAP consists of vertically oriented macular atrophy with multilobular borders, pseudodrusen-like deposits across the posterior pole and mid-periphery, and peripheral pavingstone degeneration. Nonetheless, recent research has portrayed EMAP as a highly stage-dependent condition, allowing the identification of novel disease hallmarks, including a diffuse separation between the Bruch’s membrane and the retinal pigment epithelium, along with consistent sparing of a region temporal to the macula. Additionally, retinal electrophysiology is particularly useful in distinguishing EMAP from age-related macular degeneration (AMD).

中文翻译：

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泛发性黄斑萎缩伴假性玻璃膜疣样外观 （EMAP） 临床特征、诊断标准以及来自遗传性视网膜疾病和年龄相关性黄斑变性的见解


2009 年，具有假性玻璃膜疣样外观的广泛性黄斑萎缩 （EMAP） 在法国首次被描述为一种对称且快速进展的黄斑萎缩形式，主要影响中年人。尽管最近在意大利和世界范围内发现了大量病例，但 EMAP 仍然是一种未被充分认识的疾病。EMAP 典型的临床三联征包括具有多叶缘的垂直性黄斑萎缩、横跨后极和中缘的假玻璃膜疣样沉积物以及外周铺路石变性。尽管如此，最近的研究将 EMAP 描绘成一种高度分期依赖性的疾病，可以识别新的疾病标志，包括 Bruch 膜和视网膜色素上皮之间的弥漫性分离，以及黄斑时间区域的一致保留。此外，视网膜电生理学在区分 EMAP 与年龄相关性黄斑变性 （AMD） 方面特别有用。