A Case of Idiopathic Acute Exudative Polymorphous Vitelliform Maculopathy

Abstract

Acute exudative polymorphous vitelliform maculopathy is a recently described rare disease that consists of vitelliform lesions and serous macular detachment. Other disorders, including the bestrophinopathies and paraneoplastic polymorphous retinopathy, may share the same vitelliform lesions as acute exudative polymorphous vitelliform maculopathy and could cause potential confusion in attaining the diagnosis. Here we present a case of a 22-year-old male who presented with fundoscopic examination findings of vitelliform lesions that were consistent with the aforementioned disorders. Consequently, the patient underwent an extensive diagnostic work-up, including BEST1 gene testing, electroretinogram/electrooculogram testing, and a paraneoplastic etiology testing, all of which returned negative. With the result of this work-up, the most likely diagnosis for this patient is acute exudative polymorphous vitelliform maculopathy. Given that a number of disorders present with findings of vitelliform lesions, it is important for the clinician to remain cautious and to keep acute exudative polymorphous vitelliform maculopathy in the differential diagnosis. In summary, acute exudative polymorphous vitelliform maculopathy is a rare disease consisting of vitelliform lesions and serous macular detachment that can look similar to other inheritable or acquired retinopathies. Due to this, it is vital to perform thorough testing and evaluation to rule out other potential causes of these examination findings.