Best's disease primarily affects which layers in the retina?

Best's disease, also known as Best macular dystrophy, is a genetic condition that specifically affects the retinal pigment epithelium (RPE) and Bruch's membrane. The presence of a lipid-containing pigmentary deposit in the RPE is a hallmark of this condition, and the degeneration of RPE cells significantly influences the progression of the disease. The RPE is crucial for maintaining the health of photoreceptors in the retina by providing essential support and waste removal functions, while Bruch's membrane serves as a barrier that separates the RPE from the choroidal blood supply. Damage to these layers can lead to impaired vision and the development of choroidal neovascularization (CNV) as the disease progresses. Understanding the involvement of the RPE and Bruch's membrane is crucial for recognizing the pathophysiological processes in Best's disease and for guiding management and treatment strategies.