Epidemiology, Associations, and Treatment of Acute Exudative Polymorphous Vitelliform Maculopathy (AEPVM)

Acute Exudative Polymorphous Vitelliform Maculopathy (AEPVM) is a rare retinal disorder characterized by serous subretinal detachments that progress to vitelliform lesions. In this review, we aim to summarize current knowledge of etiologies and treatments for AEPVM and its association with primary and recurrent melanoma. We performed a comprehensive search of scientific literature for articles mentioning Acute Exudative Polymorphous Vitelliform Maculopathy including all literature published through March 2025 using the online electronic databases PubMed, MEDLINE, and Embase. We identified 81 cases (mean age 46 ± 16.9 years, 43.2% female) with etiologies including idiopathic (43), infectious (10), paraneoplastic (19), and medication-induced (9). Several successful treatments were identified. Local or systemic corticosteroids in 17 eyes improved subretinal fluid and vision in 100% of idiopathic, 50% of infectious, and 40% of medication-induced cases. In paraneoplastic AEPVM, fluid improved with immunotherapy (2 eyes), chemotherapy (2 eyes), intravitreal methotrexate (1 eye), and aflibercept (2 eyes). Notably, AEPVM was the initial symptom of primary or metastatic melanoma in 5 cases. The variable response to treatment and the potential link between AEPVM and primary and recurrent melanoma highlights the need for further research. A thorough understanding of treatment options and outcomes is essential to optimize management and to encourage prompt recognition of metastatic melanoma or other conditions associated with AEPVM.