Presumed acute posterior multifocal placoid pigmentary epitheliopathy associated with Bartonella infection / Epiteliopatia pigmentar placoide multifocal posterior aguda presumível associada com infecção por Bartonella

ABSTRACT To report a unique case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in a patient with positive serology for Bartonella, presenting with ocular signs and symptoms not attributable to other diseases. A 27-year-old woman presented with decreased visual acuity in both eyes. Multimodal fundus image analysis was performed. A color fundus photograph of both eyes revealed peripapillary and macular yellow-white placoid lesions. The fundus autofluorescence of both eyes demonstrated hypo- and hyperautofluorescence of the macular lesions. Fluorescein angiography showed early-stage hypofluorescence and late staining of placoid lesions in both eyes. Spectral domain optical coherence tomography (SD-OCT) of both eyes revealed irregular elevations in the retinal pigment epithelium with the disruption of the ellipsoid zone on the topography of macular lesions. At 3 months after the treatment initiation for Bartonella infection, the placoid lesions became atrophic and hyperpigmented, and SD-OCT revealed loss of both the outer retinal layers and retinal pigment epithelium on the topography of macular lesions in both eyes.

RESUMO Caso de epiteliopatia pigmentada placoide multifocal posterior aguda presumida em paciente com sorologia positiva para Bartonella. Paciente feminina de 27 anos apresentou diminuição da acuidade visual em ambos os olhos. Análise multimodal de imagem foi realizada. A retinografia mostrou revelou lesões placoides amarelo-esbranquiçadas nas áreas peripapilar e macular de ambos os olhos. A autofluorescência demonstrou hipo e hiperautofluorescência em ambos os olhos, na mesma topografia das lesões detectadas na retinografia. A angiofluoresceínografia mostrou hipofluorescência na fase inicial do exame e hiperfluorescência tardia das lesões placoides em ambos os olhos. A tomografia de coerência óptica de domínio espectral de ambos os olhos revelou elevações irregulares do epitélio pigmentado da retina com descontinuação da zona elipsoide na área macular. Três meses após o início do tratamento para infecção por Bartonella, as lesões placoides tornaram-se atróficas e hiperpigmentadas, e a tomografia de coerência óptica revelou perda das camadas externas da retina e do epitélio pigmentado da retina na topografia das lesões maculares em ambos os olhos.

A Case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy Following Human Papilloma Virus Vaccination

PURPOSE: To report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) that developed after human papilloma virus (HPV) vaccination. CASE SUMMARY: A 31-year-old woman experienced sudden bilateral blurred vision and paracentral scotomas four days before visiting the hospital. The symptoms occurred two weeks after the second vaccination with quadrivalent HPV vaccine (Gardasil®, MSD Korea, Seoul, Korea), and she had no pain or cold symptoms. At initial visit, the anterior chamber cells were observed, and the anterior vitreous was found to be clear. On fundus examination, multiple placoid yellow-whitish lesions were observed at the posterior pole, and fluorescein angiography showed early hypofluorescence and late hyperfluorescence with staining. Under a diagnosis of bilateral APMPPE, oral corticosteroid was used as treatment for two weeks, after which symptoms were resolved, anterior chamber cells disappeared, and lesions at the posterior pole markedly decreased. After five weeks, multiple hyperreflective areas on the outer retinal layers as well as missing photoreceptor and retinal pigment epithelial layers were almost recovered upon optical coherence tomography. CONCLUSIONS: Non-infectious uveitis such as APMPPE can rarely present after HPV vaccination. Further studies are necessary to understand whether HPV vaccine is a direct cause of uveitis.

Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy

BACKGROUND AND PURPOSE: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease that causes acute visual symptoms with characteristic ophthalmoscopic findings. Neurological complications are rarely reported in the literature. Here we report two new cases of APMPPE that presented with neurological manifestations, one of which was associated with peripheral neuropathy, which has not been described before. METHODS: A retrospective database review of all patients with a diagnosis of APMPPE was performed. Clinical, ophthalmological, and neurological data were analyzed, and only cases of APMPPE with neurological complications were included. A literature review of several databases was also performed, and previous case reports were reviewed and analyzed in detail. RESULTS: In total, 56 cases of APMPPE-associated neurological complications were included in the analyses: 54 from the literature and 2 from our own practice. The most common complication was cerebral vasculitis, which affected 28 patients (50%), followed by headaches in 15 patients (26.8%). The other complications include sixth-cranial-nerve palsy, transient hearing loss, meningoencephalitis, cavernous sinus thrombosis, and viral meningitis. CONCLUSIONS: This report adds to the literature of a novel association of APMPPE with peripheral neuropathy, and comprehensively reviews the neurological manifestations of this disease. A high level of suspicion should be applied when dealing with a case of APMPPE. We recommend applying detailed clinical neurological examinations and magnetic resonance imaging to APMPPE patients, and then early steroid treatment if the examination is positive or even suspicious. Early treatment with steroids and long-term treatment with immunosuppressive azathioprine with interval neurological evaluations will contribute positively to the outcomes and avoid fatal complications, namely strokes.