Spatio-temporal understanding of the pathology of acute posterior multifocal placoid pigment epitheliopathy

We report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and the associated spatio-temporal pathologic findings of this disease.

A 29-year-old woman with bilateral recurrent APMPPE.

The patient complained of bilateral blurred vision. The best-corrected visual acuity was 20/13 bilaterally. Slit-lamp examination revealed bilateral, multiple, yellowish-white, flat, placoid lesions over the posterior fundus. A hyperreflective area between the outer nuclear layer (ONL), and the photoreceptor inner and outer segments (IS/OS) visible on the optical coherence tomographic images and a hyperfluorescent area identified on the late-phase fluorescein angiographic images were spatio-temporally consistent with the presence of the placoid lesions. The pattern of juxtafoveal hyperfluorescent spots was accompanied by multiple hypofluorescent, wedge-shaped lesions. The hypofluorescent lesions visible on the indocyanine green angiographic (ICGA) images were irregular in shape and intensity. APMPPE was diagnosed, and the patient was treated with an initial 30 mg dose of oral prednisolone, followed by a 3-week taper. The lesions recurred in an area where hypofluorescent lesions had not occurred previously, while the previous hypofluorescent spots resolved completely without the hyperfluorescence associated with vascular remodeling.

These findings suggest that the placoid appearance of APMPPE may correspond to edema between the ONL and the photoreceptor IS/OS and that the hypofluorescent lesions characteristic of this condition visible on ICGA images might reflect infiltration and subsequent blockage of choroidal vasculature-derived fluorescence.