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6: Inflammatory Disease : Acute Multifocal Placoid Pigment Epitheliopathy



Acute multifocal placoid pigment epitheliopathy (AMPPE) affects young patients who present with rapid vision decline and develop multifocal gray-white pigment epithelial lesions of the posterior pole in one or both eyes. These placoid lesions may be associated with vitreous cells, and many patients give a history of associated systemic viral illness. The disease is self-limited, requires no therapy, and resolves rapidly with an excellent visual outcome.


CASE 1

The color photograph (Figure 6-1A) demonstrates multi-focal, fuzzy, white, single and confluent discoid areas of opacified retinal pigment epithelium (RPE). Note the retinal vessel anterior to a placoid lesion (arrow). Xanthophyll within the foveal avascular zone (FAZ) appears abnormally prominent.

The early venous phase of the stereo fluorescein angiogram (Figure 6-1B) demonstrates irregular, patchy blockage of background choroidal fluorescence localized at the level of the RPE. The pattern may resemble an irregular, large spot-size, panretinal photocoagula-tion pattern. There is no evidence of sensory elevation of the retina.

The late venous phase of the stereo fluorescein angiogram (Figure 6-1C) shows the areas of RPE blockage to be slightly less prominent with subtly increasing fluorescence as some dye begins to penetrate the lesions from the posterior choroid.

Late recirculation phase photographs (Figure 6-1D) confirm a flat retina with late staining of the damaged RPE in the areas of initial blockage of background choroidal fluorescence. These multisized, irregular, hyperfluorescent spots are typical of the late-staining pattern evident in AMPPE. At this stage, the dye has stained the affected RPE cells, choroidal tissue, and, perhaps, the sclera, causing the lesions to hyperfluoresce. As these areas heal, they tend to become depigmented with scattered focal hyperpigmentation of the RPE.

fig. 6-1a
A

fig. 6-1b
B

fig. 6-1c
C

fig. 6-1d
D

FIGURE 6 - 1


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