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Placoid Retinopathies: Acute Posterior Multifocal Placoid Pigment Epitheliopathy, Serpiginous Choroiditis, Relentless Placoid Chorioretinitis, and Persistent Placoid Maculopathy

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Albert and Jakobiec's Principles and Practice of Ophthalmology

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Abstract

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE), serpiginous choroiditis (SPC), relentless placoid chorioretinitis (RPC), and persistent placoid maculopathy (PPM) are grouped together due to the “placoid” nature of their lesions. They historically fall under a broader category of white spot diseases; however, their placoid lesions and time course often distinguish them from other entities. Historically, the pathophysiology of the lesions was not entirely understood, with a controversy of the primary site being the choroid or outer retina. Multimodal imaging has been instrumental in gaining insight to these processes and revealing a primary role of choroidal flow impairment. The role of autoimmunity remains uncertain.

The placoid diseases resemble each other on clinical examination and imaging. Clinically, they are characterized by placoid outer retinal lesions. There is variable development of atrophy that is most severe in SPC as well as choroidal neovascularization, which occurs more commonly in SPC and PPM. There is evidence of choriocapillaris nonperfusion that corresponds with outer retinal changes along with some element of blockage of fluorescence. Optical coherence tomography shows backscatter and hyperreflectivity with loss of transparency of the outer nuclear layer and later development of thinning, loss of photoreceptors, and RPE damage that tends to be more advanced in SPC and PPM.

These entities can be differentiated by their time course, laterality, age distribution, and severity of permanent damage. APMPPE typically presents with a shorter disease course, while RPC confers a much more protracted course. In cases of foveal involvement, outcomes may vary with APMPPE having the most favorable prognosis and SPC carrying the poorest. All seem to respond to immunosuppression.

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Acknowledgments

Strutha C. Rouse II, MD, and Charles J Bock Jr., MD, assisted with the earlier versions of the chapter on SPC.

Evica Simjanoski, CRA, and the retina photographers at Northwestern Medicine contributed to the imaging in this chapter.

The present work was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc., New York City, and a grant from Kevin Hitzeman and Mary Dempsey.

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  1. Department of Ophthalmology, Northwestern University, Chicago, IL, USA

    Tara J. Schaab, Rukhsana G. Mirza & Lee M. Jampol

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  1. Tara J. Schaab
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Correspondence to Lee M. Jampol .

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  1. Department of Ophthalmology, Oregon Health & Science University Department of Ophthalmology, Portland, OR, USA

    Daniel Albert

  2. Massachusetts Eye and Ear, Boston, MA, USA

    Joan Miller

  3. Department of Ophthalmology, University of Illinois at Chicago Department of Ophthalmology, Chicago, IL, USA

    Dimitri Azar

  4. Department of Ophthalmology, Harvard Medical School, Boston, MA, USA

    Lucy H. Young

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  1. Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, MA, USA

    Lucia Sobrin

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Schaab, T.J., Mirza, R.G., Jampol, L.M. (2020). Placoid Retinopathies: Acute Posterior Multifocal Placoid Pigment Epitheliopathy, Serpiginous Choroiditis, Relentless Placoid Chorioretinitis, and Persistent Placoid Maculopathy. In: Albert, D., Miller, J., Azar, D., Young, L.H. (eds) Albert and Jakobiec's Principles and Practice of Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-319-90495-5_6-1

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