Pigmented paravenous retinochoroidal atrophy associated with Vogt-Koyanagi-Harada disease: a case report

Abstract

Background: To describe a unique case of pigmented paravenous retinochoroidal atrophy that developed several years after Vogt-Koyanagi-Harada disease. Case presentation: A 28-year-old woman presented with gradual vision loss in both eyes and nyctalopia for 2 years. Past medical history was relevant for Vogt-Koyanagi-Harada disease since the age of 19 and positive HLA-DR4. Funduscopic examination revealed perivascular pigmentary clumping and atrophic changes radiating from the optic disks. Spectral domain optical coherence tomography through the macula demonstrated perifoveal outer retinal layers loss with cystic degeneration. Fundus autofluorescence showed zonal areas of hypoautofluorescence corresponding to the areas of atrophy. Full-field electroretinogram identified mildly reduced scotopic and photopic responses. The patient was diagnosed with pigmented paravenous retinochoroidal atrophy. Conclusions: Pigmented paravenous retinochoroidal atrophy may be acquired after Vogt-Koyanagi-Harada disease. Pathogenesis of pigmented paravenous retinochoroidal atrophy may involve inflammatory-related precursors on a background of genetic predisposition.

WITHDRAWN: Pigmented paravenous retinochoroidal atrophy caused by VPS13B gene mutations: a case report

“…in ammatory responses [14], infectious [15,16], autoimmunity [17] Those with lower scores (⩽5 of 8) were considered provisionally to have a Cohen-like syndrome [4]. Most reported cases indicate that ocular anomalies are common among patients with Cohen syndrome.…”

Unilateral pigmented paravenous retinochoroidal atrophy with acute angle-closure glaucoma: a case report

“…The etiology of PPRCA remains unclear, but genetic, developmental and inflammatory causes have been postulated. PPRCA has been previously reported in association with tuberculosis, congenital syphilis, Behçet disease, measles, rubella [ 1 ] and Vogt-Koyanagi-Harada disease [ 10 ], but its relationship with concomitant diseases has not been reported. Our patient had no evidence of active or previous intraocular inflammation and denied any systematic infectious diseases.…”

Pigmented Paravenous Retinochoroidal Atrophy (PPRCA): a systematic review

“…It was also spread across different locations in the eyes, with some patients reporting atrophy "observed throughout" and others with more constricted presentations, such as "in the peripheral paravenous distribution" [26,37]. More speci c reports of pigment clumping corresponding to retinochoroidal atrophy were made in 35% of studies (N = 22), with "pigment clumps" or "lesions", along various parts of the eye, including "retinal veins" or "retinal vascular arcade" [2,[9][10][11][12]. Another reported characteristic of retinochoroidal atrophy was "shadowing corresponding to pigment clumps" [2,33,46].…”

“…3). Of those whose duration was recorded, 14% (N = 21) reported symptoms for under a year, 19.3% (N = 29) reported symptoms for 1-5 years, 8.6% (N = 13) reported symptoms for 5-10 years and 7.3% (11) reported symptoms for 10 + years.…”