Klin Monbl Augenheilkd
DOI: 10.1055/a-2538-2496
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A Case of Bilateral Acute Posterior Multifocal Placoid Pigment Epitheliopathy – Atypically with Monocular Subretinal Macular Fluid – in an Adolescent Female Patient and Resolving without Treatment

Ein Fall von spontan zurückgebildeter bilateraler akuter posteriorer multifokaler plakoider Pigmentepitheliopathie mit atypischem monokularem subretinalem Makulaödem bei einer jugendlichen Patientin
Maximilian Aria Antoninus Josef Schulze 
1   Ophthalmology, University Medical Centre Groningen, Groningen, Netherlands
2   Faculty of Medical Sciences, Rijksuniversiteit Groningen, Groningen, Netherlands
,
Marie Francine Hoogslag-Bienfait
1   Ophthalmology, University Medical Centre Groningen, Groningen, Netherlands
,
Charlotte Louise Leonarde Ingrid van Meerwijk
1   Ophthalmology, University Medical Centre Groningen, Groningen, Netherlands
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Introduction

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a form of inflammatory chorioretinopathy and can be associated with cerebral vasculitis [1], [2].

Gass was the first to describe APMPPE [3]. It occurs in young adulthood, with an average presentation age of 27 years and affects men and women about equally [4]. The estimated incidence is 0.15 cases per 100 000 [5]. APMPPE is characterized by multiple yellow to white lesions in the posterior pole of the macula, typically in both eyes [6]. Viral symptoms often precede this condition [6]. The exact cause of APMPPE remains unknown. Initially, it was thought to be a change in the retinal pigment epithelium (RPE), but nowadays it is seen as an immune-mediated condition, possibly triggered by an infection or linked to autoimmune conditions [4].

The lesions often resolve spontaneously [7], typically resulting in good visual recovery [8], but complete recovery is not assured in all cases [9]. Treatment with corticosteroids can be considered. Although there is no consensus in the literature regarding this approach, it is recommended for cases with foveal involvement [2].

The purpose of this paper is to report a conservatively managed case of bilateral APMPPE in a very young patient, presenting with atypical monocular subretinal fluid (SRF) with foveal involvement, accompanied by visual impairment.



Publication History

Received: 25 November 2024

Accepted: 06 February 2025

Article published online:
27 March 2025

© 2025. Thieme. All rights reserved.

Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany

 

  • References

  • 1 Papasavvas I, Herbort Jr. CP. Diagnosis and Treatment of Primary Inflammatory Choriocapillaropathies (PICCPs): A Comprehensive Overview. Medicina 2022; 58: 165
    CrossrefPubMedSearch in Google Scholar
  • 2 Algahtani H, Alkhotani A, Shirah B. Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy. J Clin Neurol 2016; 12: 460-467
    CrossrefPubMedSearch in Google Scholar
  • 3 Gass JDM. Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 1968; 80: 177-185
    CrossrefPubMedSearch in Google Scholar
  • 4 Testi I, Vermeirsch S, Pavesio C. Acute posterior multifocal placoid pigment epitheliopathy (APMPPE). J Ophthalmic Inflamm Infect 2021; 11: 31
    CrossrefPubMedSearch in Google Scholar
  • 5 Abu-Yaghi NE, Hartono SP, Hodge DO. et al. White dot syndromes: a 20-year study of incidence, clinical features, and outcomes. Ocul Immunol Inflamm 2017; 19: 426-430
    CrossrefPubMedSearch in Google Scholar
  • 6 Reinthal EK, Schlote T, Zierhut M. Neurologische Komplikationen bei akuter posteriorer multifokaler plakoider Pigmentepitheliopathie (APMPPE) – eine Literaturübersicht mit Kasuistik. Klin Monatsbl Augenheilkd 2001; 218: 756-762
    Thieme ConnectPubMedSearch in Google Scholar
  • 7 Gervasio K, Peck T. eds. The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease. 8th ed. Philadelphia: Wolters Kluwer Health; 2021: 369
    Search in Google Scholar
  • 8 Steptoe PJ, Pearce I, Beare NAV. et al. Clinical Outcomes of Observed and Treated Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis. Ocul Immunol Inflamm 2024; 32: 683-689
    CrossrefPubMedSearch in Google Scholar
  • 9 Fiore T, Iaccheri B, Androudi S. et al. Acute posterior multifocal placoid pigment epitheliopathy: outcome and visual prognosis. Retina 2009; 29: 994-1001
    CrossrefPubMedSearch in Google Scholar
  • 10 Raven ML, Ringeisen AL, Yonekawa Y. et al. Multi-modal imaging and anatomic classification of the white dot syndromes. Int J Retina Vitreous 2017; 3: 12
    CrossrefPubMedSearch in Google Scholar
  • 11 Vedantham V, Ramasamy K. Atypical manifestations of acute posterior multifocal placoid pigment epitheliopathy. Indian J Ophthalmol 2006; 54: 49-52
    CrossrefPubMedSearch in Google Scholar
  • 12 Wolf MD, Folk JC, Panknen CA. et al. HLA-B7 and HLA-DR2 antigens and acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol 1990; 108: 698-700
    CrossrefPubMedSearch in Google Scholar
  • 13 Xerri O, Salah S, Monnet D. et al. Untreated Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): a case series. BMC Ophthalmol 2018; 18: 76
    CrossrefPubMedSearch in Google Scholar