Acute Posterior Multifocal Placoid Pigment Epitheliopathy Sharing Characteristic OCT Findings of Vogt-Koyanagi-Harada Disease

Abstract

A 17-year-old male presented with acute bilateral paracentral scotomata and blurred vision. Funduscopic examination showed bilateral macular serous retinal detachment and yellow-white placoid lesions at the level of retinal pigment epithelium. OCT study showed typical VKH disease findings with marked choroidal thickening and macular serous retinal detachment partly with subretinal septa in both eyes. FA demonstrated hypofluorescence at the placoid lesions in the early phase and hyperfluorescence in the late phase. Laboratory investigation showed negative result for HLA-DR4 serotype and the patient’s cerebrospinal fluid test values were within normal range. We made the diagnosis of APMPPE from these results. At 2-month follow-up without the use of corticosteroids, OCT reexamination showed complete amelioration of subretinal fluid in both eyes. Patchy pigmentary lesions also resolved clinically with partial chorioretinal scars. The results in this case suggested OCT findings in APMPPE patients could be similar to characteristic features usually found in acute VKH disease. We recommend comprehensive assessments such as FA, cerebral spinal fluid analysis, and HLA typing which help in leading proper diagnosis.

Proposing a Neurotropic Etiology for Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Relentless Placoid Chorioretinitis

“…Areas of ONL hyperreflectivity following the HFL, identical to those demonstrated in APMPPE (Figure 1) are also seen in acute macula neuroretinopathy (49) and hyperreflective dots following the HFL have been demonstrated in MEWDS (61). While the appearance of bacillary layer detachments is most commonly associated with cases of VKH, the appearance has also been reported in cases of APMPPE (4,(62)(63)(64) and observed in our series (Supplementary Figure 4). As we have hypothesised that areas of hypofluorescence on indocyanine green angiography occur as a consequence of RPE-BM separation (Figure 6), areas of RPE-BM separation can also be observed in early bacillary layer detachments (Supplementary Figure 4).…”

Acute posterior multifocal placoid pigment epitheliopathy associated with serous retinal detachment: A case report

“…According to Kitamura and al, indication of treatment in APMPPE is not quite established. Resolution of SRD suggest that there is a spontaneous cessation of choroidal inflammation [ 10 ]. Therefore, SRD is not an absolute indication for corticosteroid treatment in APMPPE.…”

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)

“…Accumulation of associated subretinal fluid might occur in APMPPE, and the presence of exudative retinal detachment mimicking acute Vogt-Koyanagi-Harada has been reported [44,45]. Recently, Kohli et al described three cases of APMPPE characterized in the acute phase by a SD-OCT revealing a splitting of the ellipsoid zone suggestive of bacillary layer detachment [46].…”

“…During the acute phase of the disease enhanced depth imaging optical coherence tomography (EDI-OCT) shows choroidal thickening, improving with the resolution of the placoid lesions (Fig. 2) [44][45][46].…”