A Case of Neurosyphilis Revealed by Acute Exudative Polymorphous Vitelliform Maculopathy

Introduction

Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare disorder involving the retina and the retinal pigment epithelium (RPE) first described by Gass in 1988.¹ The funduscopic findings are distinctive, with multifocal post-equatorial small discrete round or oval shallow retinal detachments with yellowish subretinal lesions. Gass originally reported two patients with acute headaches and bilateral vision loss. One of them had biomicroscopic signs of intraocular inflammation with a few vitreous cells, and both responded well to corticosteroids.¹ AEPVM can be either idiopathic or paraneoplastic but has never been reported to be associated with infectious etiologies in the published literature. We report a case of neurosyphilis revealed by typical AEPVM.

Case Report

A healthy 56-year-old man presented with a 3-week history of bilateral vision loss. Visual acuity was 20/25 in both eyes. The anterior segment was unremarkable. Funduscopic examination revealed a few vitreous cells and multifocal discrete post-equatorial shallow retinal detachments with yellowish lesions arranged in a pseudo-hypopion configuration in the posterior pole in both eyes (Figure 1). The yellowish vitelliform lesions appeared hyperautofluorescent and moderately hypofluorescent on fluorescein angiography (FA) by blocking defect. Spectral-domain optical coherence tomography (SD-OCT) revealed multiple serous retinal detachments with diffuse deposition of hyperreflective material at the posterior border of the neurosensory retina and above the RPE band (Figure 1). There was also a diffuse thickening of the outer segments of the photoreceptors more widespread than the retinal detachments on SD-OCT (Figure 1). The clinical, angiographic, and SD-OCT findings were typical of the diagnosis of AEPVM, and the patient was referred for a complete systemic evaluation for cancer.

Figure 1. Multimodal imaging of both eyes of a 56-year-old man who presented with a bilateral decrease in visual acuity to 20/25. Color fundus photographs of both eyes (A, B) demonstrate bilateral multifocal discrete post-equatorial shallow retinal detachments with yellowish lesions arranged in a pseudo-hypopion configuration in the posterior pole. The yellow deposits correspond to hyperautofluorescent lesions in the fundus autofluorescence (FAF) images (C, D) and to slightly hypofluorescent lesions on the fluorescein angiography (FA) images (E, F). Note there is no leak point or staining of the vessels or the optic disc on FA (E, F). There are multiple serous retinal detachments, hyperreflective vitelliform lesions, and diffuse thickening of the outer segments of the photoreceptors on spectral-domain optical coherence tomography horizontal (G, H) and vertical (I, J) foveal scans.

He returned 10 days later with new complaints of bilateral redness, pain, and vision loss in both eyes. Anterior segment examination revealed bilateral granulomatous anterior uveitis, and visual acuity had decreased to 20/80 in the right eye and 20/40 in the left eye. An inflammatory work-up yielded a positive titer of treponemal antibodies in the serum and cerebrospinal fluid, indicating a neurosyphilis infection. There was no co-infection with human immunodeficiency virus and no other systemic clinical finding. The patient received a standard treatment for neurosyphilis with intravenous penicillin G at a dose of 24 million units per day for 15 days along with topical corticotherapy in both eyes. Two months later, the intraocular inflammation had resolved and visual acuity had improved to 20/25 in both eyes. Funduscopic examination revealed the resolution of the serous component of the retinal detachments. The resolution of the vitelliform lesions was more gradual with a concomitant rearrangement in a ring configuration (Figures 1 and 2).

Figure 2. Multimodal imaging of both eyes 2 months after initiation of intravenous antibiotherapy. Color fundus photographs of both eyes (A, B) demonstrate the persistence and rearrangement in a ring configuration of the multifocal vitelliform lesions that appear hyperautofluorescent in the fundus autofluorescence images (C, D). The spectral-domain optical coherence tomography horizontal foveal scans show resolution of the retinal serous detachments and the persistence of some vitelliform lesions subfoveally (E, F).

Discussion

Acquired syphilis is a chronic sexually transmitted disease caused by the spirochete Treponema pallidum. The protean clinical presentations of syphilis lent it the name of the “great mimicker.”

Acute syphilitic posterior placoid chorioretinitis was first described by Gass in 1990² as a placoid whitened lesion located in the posterior pole. FA usually demonstrates staining of the outer retina, and OCT may reveal³ at the acute phase subretinal fluid, disruption of the ellipsoid zone, and hyperreflective thickening of the RPE. The outer retinal abnormalities typically resolve after treatment. Punctate hyperautofluorescent spots may be observed in few cases.³

This 56-year-old man first presented with clinical features consistent with the diagnosis of AEPVM. He returned 10 days later with a bilateral anterior granulomatous uveitis, and the inflammatory work-up revealed treponemal antibodies in the serum and spinal fluid consistent with a diagnosis of active neurosyphilis. AEPVM was first reported to be either an identified genetic disorder or an acquired condition, possibly virus-induced.^1,4 Gass reported that the clinical and angiographic features were consistent with a diffuse abnormality of the RPE.¹ We observed a diffuse thickening of the outer segments more widespread than the multiple shallow retinal detachments on SD-OCT, consistent with this hypothesis. It is possible that there is a diffuse RPE dysfunction responsible for improper phagocytosis of the outer segments of the photoreceptors, with subsequent diffuse thickening of the outer segments and multifocal accumulation of vitelliform lesions.

Although histopathologic analysis is not available, the typical large placoid yellowish lesions of acute syphilitic posterior placoid chorioretinitis have been postulated to be the result of an active inflammatory reaction at the level of the RPE-photoreceptor complex.³ Therefore, an immune process involving antibodies targeting the RPE cells with subsequent RPE dysfunction could be a plausible explanation for the clinical findings of AEPVM in our patient.

Clinicians should be aware that syphilis can produce AEPVM and should look carefully for inflammatory vitreous and anterior chamber cells. Syphilitic chorioretinitis should be considered in the differential diagnosis of AEPVM.