A Review of the Inflammatory Chorioretinopathies: The White Dot Syndromes

The white dot syndromes are a group of inflammatory chorioretinopathies of unknown etiology which have in common a unique and characteristic appearance of multiple yellow-white lesions affecting multiple layers of the retina, retinal pigment epithelium (RPE), choriocapillaris, and the choroid. They also have overlapping clinical features. We discuss acute retinal pigment epitheliopathy, multiple evanescent white dot syndrome, acute posterior multifocal placoid pigment epitheliopathy, multifocal choroiditis and panuveitis, acute zonal occult outer retinopathy, birdshot chorioretinopathy, and serpiginous choroidopathy. Some of these diseases are associated with a viral prodrome suggesting a possible viral/infectious etiology, while others are associated with a number of systemic processes suggesting an autoimmune etiology. We also review the presentation, evaluation/diagnosis, and treatment of these entities as well as the prognosis. Where applicable we discuss recent advancements in diagnosing and treating the white dot syndromes. 1. Introduction White dot syndromes is a term that has come into use over time to describe a group of inflammatory chorioretinopathies, not necessarily related to each other in pathogenesis, management, or prognosis. The common feature is a superficial resemblance of the lesions to each other. Nevertheless, the term conjures in the minds of readers a group of diseases and their unique features. Inflammatory chorioretinopathies, referred to as “white dot syndromes,” are of unknown etiology and typically affect young, healthy adults. Common presenting symptoms include photopsias, blurred vision, floaters, and visual field loss [1]. Many syndromes are characterized by a viral prodrome. The presence of discrete white lesions are located at various levels of the retina, outer retina, RPE, choriocapillaris, and choroid depending on the class of white dot condition [1]. While the etiology of white dot syndromes remains unknown, some suggest an autoimmune/inflammatory cause triggered by an exogenous agent. Some debate that the white dot syndromes are a spectrum of the same disease process, while others debate that each is a separate disease entity [2]. Despite the white dot similarities, most can be distinguished by the natural history, lesion morphology and progression, and fluorescein angiography pattern. 2. Acute Retinal Pigment Epitheliitis (ARPE, Krill Disease) Acute retinal pigment epitheliitis (ARPE) is a unilateral condition that occurs in otherwise healthy young adults in the second to fourth decade. Typically, this benign and