Untreated Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): a case series

Olivia Xerri,Sawsen Salah,Dominique Monnet,Antoine P Brézin

doi:10.1186/s12886-018-0744-z

Abstract

BackgroundAcute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory eye disease that affects the Retinal Pigment Epithelium and outer retina. The purpose of this study was to describe its presentations, as well as its prognosis in a series of untreated patients.MethodsRecords of patients seen in the department of Ophthalmology at Cochin University Hospital, Paris, between April 2002 and June 2015 were retrospectively studied. Patients were included if they presented with the typical findings of APMPPE characterized by whitish or yellowish bilateral placoid lesions, a typical pattern of early hypofluorescence and late hyperfluorescence on fluorescein angiography. Only untreated patients who had been followed for at least 1 month were included.ResultsOut of 22 patients’ records with a diagnosis of APMPPE, 10 patients (9 women, 1 man), with a mean age of 24.5 ± 4.2 years, fulfilled the study criteria with a diagnosis of typical untreated APMPPE. Prodromal symptoms were reported in 7/10 patients. Macular lesions were observed in 18/20 eyes. Sub-retinal fluid was seen at presentation in 3 eyes. Initial mean BCVA was 0.56 ± 0.81 LogMAR [− 0.10 to 2.30]. In 9 out of 10 cases, the time interval between manifestations in the first affected eye and the fellow eye was less than 3 days. After 1 month, BCVA had improved to 0.05 ± 0.089 LogMAR [0–0.3], with a decimal BCVA ≥0.8 in 17/20 eyes.ConclusionsIn these 10 cases of untreated APMPPE, a favorable outcome was observed.

Highlights

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory eye disease that affects the Retinal Pigment Epithelium and outer retina
The purpose of our study was to assess the ocular and extra-ocular features, as well as the visual prognosis in a group of untreated patients. This was a retrospective study of patients with a diagnosis of APMPPE seen between April 2002 and June 2015 in the department of Ophthalmology of the Cochin University Hospital, Paris
Our analyses included the following parameters assessed at entry and at the 1 month-follow-up: best-corrected-visual-acuity (BCVA), results of slit-lamp examination, Humphrey visual field testing and fundus imaging by fluorescein angiography

Summary

Introduction

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare inflammatory eye disease that affects the Retinal Pigment Epithelium and outer retina. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare inflammatory disease that typically affects healthy young adults. Five decades after its first description, it remains debated whether the primary tissue involved is the choriocapillaris or the retinal pigment epithelium [1,2,3,4]. Patients typically present with a rapid onset of visual loss associated with central and paracentral scotomas. The observation of the fundus typically shows multifocal, yellowish-white, placoid lesions, varying in size, located from the posterior pole to the mid-periphery. The lesions show early hypofluorescence and late hyperfluorescence (“blocks early, stains late”). The lesions fade gradually within weeks, to be replaced by varying degrees of hyperpigmentation and sometimes by retinal pigment epithelium (RPE) atrophy

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