Acute posterior multifocal placoid pigment epitheliopathy presenting with granulomatous panuveitis

To describe a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) presenting with granulomatous panuveitis and to highlight multimodal ocular imaging findings useful for diagnosis.

A 20-year-old Japanese man presented with acute vision loss in the left eye. Best-corrected visual acuity was 20/20 in the right eye and 20/250 in the left eye. Slit-lamp examination revealed bilateral mutton-fat keratic precipitates with anterior chamber cells and anterior vitreous cells. Fundus examination showed multiple confluent yellowish-white placoid lesions bilaterally. Angiographic and OCT findings were characteristic of APMPPE. These included the angiographic reversal phenomenon on fluorescein angiography, persistent hypofluorescence on indocyanine green angiography, and the angular sign of Henle fiber layer hyperreflectivity on optical coherence tomography. Systemic evaluation, including syphilis serology, an interferon-gamma release assay for tuberculosis, serum angiotensin-converting enzyme and soluble interleukin-2 receptor levels, viral serology, and chest radiography, did not identify an associated infectious or inflammatory disease. Bilateral sub-Tenon triamcinolone acetonide injections, oral prednisolone, and topical corticosteroids led to rapid resolution of inflammation and recovery of visual acuity. The patient remained free of recurrence for 13 months after presentation, including more than seven months after cessation of therapy.

APMPPE should remain in the differential diagnosis of granulomatous panuveitis when placoid fundus lesions are present. Careful ocular imaging and systemic evaluation are useful for distinguishing APMPPE from VKH disease, sarcoidosis, and infectious uveitis.