Pseudoretinitis pigmentosa due to syphilis: a case report and literature review

Abstract

Syphilis is a sexually transmitted infection caused by the spirochete Treponema pallidum. Ocular involvement can occur at any time, and it may affect 10% of patients in the secondary stage, and from 2% to 5% in the tertiary stage. Uveitis is the most common presentation of ocular syphilis, affecting 0.4% to 8% of patients with systemic disease. Chorioretinitis is the most common posterior alteration. We present the case of a 53-year-old male patient, presenting with bilateral low visual acuity and nyctalopia for 3 years. His physical examination revealed decreased pupillary reflex, anterior vitreous cells, physiologic papillae, arteriolar attenuation, reduced foveal reflex, diffuse retinal pigment epithelium atrophy, peripapillary and perivascular punctate pigment accumulation and peripheral chorioretinitis. Full-field electroretinogram was extinct in both eyes. Treponemal syphilis test was positive. Previously diagnosed as retinitis pigmentosa, evolved to blindness, despite proper treatment. Our case shows syphilis as a significant cause of blindness. Atypical presentations of retinitis pigmentosa must warn ophthalmologists to etiologies of pseudoretinitis pigmentosa, such as syphilis.

Retinitis Pigmentosa Masquerades: Case Series and Review of the Literature

“…Examination revealed anterior vitreous cells, arteriolar attenuation, diffuse RPE atrophy, and retinal pigmentary changes bilaterally, with extinguished ffERG readings in both eyes. Initially presumed to have RP, this patient was later diagnosed with syphilis, as confirmed by a positive FTA-Abs immunoglobulin G test [24]. In contrast to patients with RP, patients with ocular syphilis may present with a unilateral or asymmetric bilateral patchy chorioretinitis that can rapidly progress, culminating in chorioretinal scars in late stages [25].…”

Pigmented Paravenous Retinochoroidal Atrophy (PPRCA): a systematic review

“…It is often found to be "consistent with retinal pigment epithelium degeneration", present along the retinal veins and choroidal vessels [2,9,18,[33][34][47][48][49]. Some studies also reported "hyper uorescence rings", with one case series of four patients reporting demarcation lines too [47,[51][52][53][54]. A window defect is an area of hyper uorescence, arising when the retinal pigment epithelium (RPE) is damaged and there is an absence of pigmentation.…”

“…The full-eld electroretinogram (ffERG) is an electrophysiologic test, assessing retinal functioning through exposure to a full-eld light-stimulus. Eleven studies reported decreased scotopic and photopic waves [3,11,18,20,23,25,32,46,52,60], whilst 7 studies reported decreased a-and b-wave amplitudes [3,11,18,20,23,46,52]. Damaged or dysfunctional photoreceptors of rods and cones were found in a few PPRCA patients [16,34,58].…”