A case of acute posterior multifocal placoid pigment epitheliopathy demonstrating vogt-koyanagi-harada disease-like optical coherence tomography findings in the acute stage

Abstract

Purpose: We report a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE), which was difficult to differentiate from posterior pole-type Vogt-Koyanagi-Harada (VKH) disease because the lesions were mainly located in the macula bilaterally.

Case report: A 33-year-old man presented with rapid bilateral loss of vision. Fundoscopy revealed yellow-white subretinal lesions in the posterior pole of both eyes. Optical coherence tomography (OCT) revealed the presence of subretinal fluid with a subretinal septum. After initiation of systemic steroids, OCT revealed that the amount of subretinal fluid decreased immediately. However, vision loss was less responsive to the therapy, and OCT revealed partial reorganization of the inner segment/outer segment (IS/OS) line in the bilateral macular areas after therapy.

Discussion: In our case, the location of the macular lesions made it difficult to differentiate APMPPE from VKH disease by fluorescein angiography. OCT images showed VKH disease-like findings of serous retinal detachment with a subretinal septum. The outer nuclear layer disappeared and the IS/OS line in the affected area was disorganized in the acute stage of the disease. In this case, the rapid loss of vision was specific to the onset pattern of APMPPE, and the slow response to therapy was very different from the response typically observed in VKH disease. Thus, careful consideration of the clinical course is important for diagnosing APMPPE.

Keywords: Acute posterior multifocal placoid pigment epitheliopathy; Optical coherence tomography; Pigment epitheliopathy; Vogt-Koyanagi-Harada disease.

Fig. 1

Changes of BCVA throughout the disease course. Day −3 was the day the patient first visited our hospital, and it indicates the third day before the initiation of systemic steroid therapy. Day 0 indicates the first day of systemic steroid therapy. Day 11 indicates the 11th day after initiation of systemic steroid therapy. BCVA OD and BCVA OS indicate BCVA of the right and left eye, respectively.

Fig. 2

Fundus photographs taken on day −1. Day −1 indicates the day before the initiation of systemic steroid therapy. a, b Fundoscopy revealing yellow-white lesions in the posterior pole of both eyes. The lesions are mainly located in the macular area bilaterally. c, d Apparent hypofluorescence is unremarkable in the early phase of FA.

Fig. 3

a, b SD-OCT images on day −1. Day −1 indicates the day before the initiation of systemic steroid therapy. SD-OCT images of both eyes showing serous retinal detachment with a subretinal septum and an intraretinal compartmentalized cystic space. The compartmentalized intraretinal space was observed to contain two hyperreflective membranous layers (white arrow). In the left eye, PED was observed (red arrow). c, d On day 1, SD-OCT revealed that the subretinal fluid began to decrease. Disruption of the hyperreflective membranous layers in the intraretinal spaces (orange arrow) was observed. Day 1 indicates the first day after initiation of systemic steroid therapy. e, f On day 3, the amount of subretinal fluid almost disappeared, but the IS/OS line remained disorganized in the affected area. Day 3 indicates the third day after initiation of systemic steroid therapy.

Fig. 4

a, b On day 15, OCT images showed that the IS/OS line remained disorganized, the retina appeared to be thin, and the outer nuclear layer appeared to be poorly reorganized. Day 15 indicates the 15th day after initiation of systemic steroid therapy. c, d OCT detected partial recovery of the IS/OS line in the macular area of both eyes on day 48 (white arrow). Day 48 indicates the 48th day after the initiation of systemic steroid therapy. e, f Reorganization of the IS/OS line was observed by OCT on day 111 (black arrow). Day 111 indicates the 111st day after initiation of systemic steroid therapy.