Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)

Overview

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is characterised by the presence of plaque-like lesions at the posterior pole which resolve over 1-2 weeks.

This condition affects men and women equally and typically starts off unilaterally with the second eye becoming involved a few days later. This condition resolves spontaneously without treatment and has a good prognosis unless there is recurrent disease, the fovea is affected or the patient is over 60 years of age. Large lesions may result in RPE atrophy and hyperplasia.

APMPPE can be associated with anomalous findings such as optic disc inflammation, periphlebitis, disc neovascularisation and exudative retinal detachment.

Presenting symptoms include blur, photophobia and paracentral scotoma. Clinically, cream-coloured placoid lesions are noted in the acute phase. OCT shows hyper-reflectivity of the outer retinal layer, disruption to the ellipsoid zone and/or RPE and sometimes outer retinal cysts.

As the lesion resolves, the hyper-reflectivity on OCT reduces and RPE atrophy may be noted.

Fundus autofluorescence imaging shows the placoid lesions to be hypo-fluorescent in the acute phase, becoming hyper-fluorescent as they regress.

Case Examples

Differential Diagnosis

Multifocal Evanescent White Dot Syndrome

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Birdshot chorioretinopathy

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Multifocal choroiditis/Punctate inner choroidopathy

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References

Knickelbein, JE. Nida Sen, H. (2016) Multimodal Imaging of the White Dot Syndromes and Related Diseases J Clin Exp Ophthalmol. 2016 June ; 7(3).

Wong, E. Nivison-Smith, L. Assaad, NN. Kalloniatis, M. (2015). OCT and fundus autofluorescence enhances visualization of white dot syndromes. Optometry and Vision Science, 92(5):642-53.