A CARE-compliant article: a case report of possible association between recurrence of multiple evanescent white dot syndrome and the Herpesviridae family

Abstract

Rationale: Multiple evanescent white dot syndrome (MEWDS) is a self-limited multifocal chorioretinopathy that typically affects otherwise healthy young females in the second to fourth decades of life. Current understanding of the pathophysiology of MEWDS is still limited. One of the possible underlying causes is an infectious etiology. Patient concerns: A 24-year-old female with recurrent episodes of typical MEWDS ocular manifestation was observed over 2 years. Viral-specific antibody serologic tests showed evidence of exposure to the Herpesviridae family during the acute stage of MEWDS in the first and recurrent episodes. Diagnoses: MEWDS was diagnosed by the clinical findings and ancillary testing results of fundus photography, optical coherence tomography, fluorescein angiography, indocyanine green angiography and electroretinogram. The laboratory serology data was positive for varicella-zoster virus (VZV) immunoglobulin M (IgM) in the first episode and exhibited high Epstein–Barr virus (EBV) elevated immunoglobulin G (IgG) titer in the recurrent episode. Interventions: Due to the self-limited nature of MEWDS, we observed the clinical course without intervention. Outcomes: During acute onset of MEWDS, serologic data for VZV IgM antibody was positive in the first episode. Two years later, the patient had recurrent episodes of MEWDS in the contralateral eye. Serologic study showed highly elevated IgG titer (1:160) of Epstein-Barr virus capsid antigen (EB-VCA) in the acute stage. The follow-up paired serum virus serology test showed that the prior EB-VCA IgG titer decreased fourfold to 1:40 in the recovery stage. Lessons: Recurrence of MEWDS may be associated with acute systemic infection of the Herpesviridae family or virus-induced autoimmune inflammatory reaction.

Did the COVID-19 Pandemic Increase the Incidence of Acute Macular Neuroretinopathy?

“…Similarly, MEWDS patients may also present with a history of viral infection. In fact, some cases associated with a previous infection with a neurotropic Herpesviridae family virus have been described [ 32 ]. Interestingly, compared to AMN, the incidence of MEWDS (which is an RP epithelitis [ 33 ] supposed to be associated with antigen-driven inflammatory reaction and immunity disorders) and of PAMM (the mechanism of which is believed to be ischemic, involving the hypoperfusion of DCP [ 34 ]) did not increase during the pandemic in our center.…”

Topographic patterns of retinal lesions in multiple evanescent white dot syndrome

“…It has been hypothesized that MEWDS develops from the complex interplay between genetic susceptibility and environmental triggers [ 21 ]. Environmental factors may include exposure to retinal antigens resulting from damage to the RPE-Bruch’s membrane interface, which may expose retinal antigens and trigger a local inflammatory response [ 7 , 8 ], or potential associations with viral infections such as acute Epstein-Barr virus or herpes virus infections [ 22 , 23 ]. However, the inherent problems with serum retinal autoantibody testing make it challenging to prove this theory [ 24 , 25 ].…”

Active Ocular Toxoplasmosis Associated with Simultaneous Multiple Evanescent White Dot Syndrome

“…MEWDS associated with herpes viridae family infection, 11 Hepatitis A, Hepatitis B, Influenza, Human Papillomavirus, meningococcus vaccinations and EBV infection 12 or prior ocular toxoplasmosis 13 have been reported but no association with active ocular toxoplasmosis has been described yet.…”