A Case of Acute Posterior Multifocal Placoid Pigment Epitheliopathy Demonstrating Vogt-Koyanagi-Harada Disease-Like Optical Coherence Tomography Findings in the Acute Stage
A 33-year-old man presented with loss of vision in his right eye, which had developed the day before. Best-corrected visual acuity (BCVA) measured using the Landolt ring chart was 0.03 and 1.5 in the right and left eye, respectively (fig. , day −3; 3 days prior to steroid pulse therapy). No remarkable findings or inflammation were observed in the anterior segment of either eye. Fundoscopy revealed a yellow-white inflammatory subretinal lesion in the posterior pole of the right eye. Spectral-domain optical coherence tomography (SD-OCT) showed the presence of subretinal fluid with a subretinal septum. No fundoscopy or SD-OCT was performed in the left eye at the first hospital visit. The next day, he presented with rapid loss of vision in his left eye, and BCVA in his left eye had decreased from 1.5 to 0.1 (fig. , day −2). Fundoscopy revealed a yellow-white lesion in the posterior pole of his left eye, which was similar to the lesion in his right eye (fig. ). SD-OCT images of both eyes showed serous retinal detachment with a subretinal septum and an intraretinal compartmentalized cystic space (fig. ). In his left eye, pigment epithelial detachment (PED) was observed on OCT (fig. ). The lesions in both eyes were limited to the macular area. Blood examination showed no remarkable abnormalities. Antibodies for Toxoplasma gondii were negative. Furthermore, the rapid plasma reagin test and fluorescent treponemal antibody absorption test were negative. The patient did not complain of abnormal skin sensations, headaches, hearing problems, or prodromal symptoms suggestive of viral infection. Apparent hypofluorescence was unremarkable in the early phase of FA (fig. ). Fluorescein dye in both eyes became gradually enhanced in the late phase of FA (not shown).\nSteroid pulse therapy comprising methylprednisolone (1,000 mg/day) was intravenously administered for 3 days, followed by oral prednisolone for 17 weeks (tapering from 50 mg/day for 4 days, 40 mg/day for 4 days, 30 mg/day for 4 days, 20 mg/day for 20 days, 15 mg/day for 28 days, 10 mg/day for 48 days, and 5 mg/day for 14 days). After initiation of systemic steroid therapy, SD-OCT revealed that the amount of subretinal fluid decreased immediately, but the inner segment/outer segment (IS/OS) line remained disorganized (fig. ). Recovery of visual acuity was delayed compared to the rapid decrease in subretinal fluid. Goldmann perimetry performed 1 week after initiation of systemic steroid therapy revealed that a central relative scotoma persisted in both eyes (data not shown). Fifteen days after initiation of therapy, BCVA was 0.09 in both eyes (fig. ). In addition, OCT showed that the IS/OS line remained disorganized, the retina appeared to be thin, and the outer nuclear layer seemed to be poorly organized (fig. ). Until day 33 of therapy, BCVA was <0.1 in both eyes, and it changed to 0.1 in both eyes on day 34 after therapy initiation (fig. ). The response to therapy was slow and very different from that typically observed in VKH disease. We made the diagnosis of APMPPE on the basis of the rapid loss of vision bilaterally and the classic manifestation of yellow-white lesions in the fundus []. Eventually, vision began to recover and BCVA improved to 0.4 in the right eye and 0.5 in the left eye (fig. , day 48). At this time point, OCT revealed the partial recovery of the IS/OS line in the macular area of both eyes (fig. ). On day 111 of therapy initiation, BCVA improved to 0.7 in the right eye and 0.6 in the left eye (fig. ), and OCT revealed clear reorganization of the IS/OS line (fig. ). One year after therapy initiation, BCVA remained 0.7 in the right eye and 1.0 in the left eye. However, unfortunately, we were unable to perform the OCT and examine the fundus.