Panretinal acute multifocal placoid pigment epitheliopathy: a novel posterior uveitis syndrome with HLA-A3 and HLA-C7 association

Background The purpose of this study is to report a previously undescribed clinical entity resembling acute posterior multifocal placoid pigment epitheliopathy (APMPPE) but with an atypical, panretinal and diffuse presentation in young patients with an HLA-A3 and HLA-C7 association. We describe a cluster of three, young, healthy patients who experienced acute visual loss, aged 16 through 27 years exhibiting an unusual clinical entity over an 8-month period. Findings Our patients demonstrated a unique presentation with acute retinal lesions similar to APMPPE but had widespread presentation of multiple lesions in the peripheral retina. All three of our patients exhibited an acute loss of vision, two of them bilaterally. Ophthalmoscopy and fundus photography demonstrated a diffuse distribution of lesions located extensively throughout the retina. On fluorescein angiography, the lesions showed a characteristic ‘blocking early and staining late’ pattern similar to APMPPE. The average duration of activity was 6 weeks (range 4 to 8 weeks), and there were no recurrences and good visual prognosis. HLA-A3 and HLA-C7 was noted in 100% of the patients. Ocular coherence tomography during the acute stage in one patient demonstrated thickening at the RPE layer. Conclusions To our knowledge, this cluster of young patients represents a previously undescribed clinical entity, with clinical features similar to APMPPE, relentless placoid chorioretinitis and ampiginous. Due to the diffuse distributions of the active lesions, the acute clinical course without recurrences, good visual prognosis, and HLA-A3/C7 association, we believe it to be distinct from these other white dot syndromes. All three patients experienced preceding viral-like prodrome which, when combined with major histocompatibility commonalities, may predispose these individuals to an immune response. We call this entity panretinal acute multifocal placoid pigment epitheliopathy or PAMPPE.