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Case 16


Patient Presentation: A 26-year-old woman of Hawaiian descent was referred to a tertiary uveitis clinic by her optometrist for a possible retinopathy in her right eye. The patient noted 1 week of seeing “gray patches” in her central vision OD. She denied pain, flashes, floaters of a visual field deficit. There was no viral prodrome. She denied past medical or ocular history. Review of systems was negative. Visual acuity was 20/70 OD and 20/20 OS. There was no RAPD. Anterior segment examination was unremarkable. Fundus photographs and OCT macula imaging was conducted and is shown below:


Question 1: What is the main finding in the right eye OCT macula image above?

Next, intravenous fluorescein angiography was conducted. The early and late phase IVFA of the right eye, and late phase of the left eye, are shown below:


Question 2: What is the main finding of the IVFA images above?

Question 3: Based on the patient demographics, history, fundus photos, OCT images, and IVFA results above, what is the most likely diagnosis?

Question 4: What alternative imaging modality should be ordered if a diagnosis of APMPPE is concluded?

At the 6-month follow-up, the patient’s vision was 20/20 OD and 20/20 OS. Though more lesions were seen OU at the 1-month and 2-month the follow-ups, these demonstrated hypo-autofluoresence at this time and were not thought to be active. Her OCT imaging demonstrated marked improvement in ellipsoid zone integrity OD. Her MRI/MRA brain was normal.



1. Gass JD. Acute posterior multifocal placoid pigment epitheliopathy. Arch Ophthalmol. Aug 1968;80(2):177-185.

2. Steptoe PJ, Pearce I, Beare NAV, et al. Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis. Front Ophthalmol. 2022;1:802962.


3. Scheufele TA, Witkin AJ, Schocket LS, et al. Photoreceptor atrophy in acute posterior multifocal placoid pigment epitheliopathy demonstrated by optical coherence tomography. Retina. Dec 2005;25(8):1109-1112.


4. Oliveira MA, Simão J, Martins A, Farinha C. Management of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Insights from Multimodal Imaging with OCTA. Case Rep Ophthalmol Med. 2020;2020:7049168.


5. Asano S, Tanaka R, Kawashima H, Kaburaki T. Relentless Placoid Chorioretinitis: A Case Series of Successful Tapering of Systemic Immunosuppressants Achieved with Adalimumab. Case Rep Ophthalmol. 2019 Jan-Apr 2019;10(1):145-152.

Learning Objectives:

  1. To understand the clinical presentation and prognosis in APMPPE

  2. To understand the fundus, OCT and IVFA findings in APMPPE

  3. To understand the importance of a full neurological work-up for CNS vasculitis in the setting of APMPPE

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