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K. R. Baxter, E. Mitchel Opremcak, MD; Panretinal Acute Multifocal Placoid Pigment Epitheliopathy: A Novel Posterior Uveitis Syndrome With HLA-A3 and HLA-C7 Association. Invest. Ophthalmol. Vis. Sci. 2010;51(13):5886.
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To report a previously undescribed clinical entity resemblingacute posterior multifocal placoid pigment epitheliopathy (APMPPE)but with an atypical, panretinal and diffuse presentation inyoung patients with an HLA-A3 and HLA-C7 association.
The case notes of the patients were reviewed and the clinicalfeatures are presented and discussed.
Our patients demonstrated a unique clinical presentation withacute retinal lesions similar to APMPPE, but also had a widespreadpresentation of multiple lesions in the peripheral retina. Allfour of our patients exhibited an acute loss of vision, threeof them being bilaterally. Ophthalmoscopy and fundus photographydemonstrated a diffuse distribution of lesions located extensivelythroughout the entire retina. On fluorescein angiography, thelesions showed a characteristic "blocking early and staininglate" pattern similar to APMPPE. The average duration of activitywas 4.75 weeks (range 3-8 weeks) and there were no recurrences.HLA-A3 and HLA-C7 was noted in three of the 3 consenting patients.Ocular coherence tomography (OCT) during the acute stage inone patient found thickening at the RPE/choriocapillaris layer.
To our knowledge, this cluster of young patients represent apreviously undescribed clinical entity, with clinical featuressimilar to APMPPE, relentless placoid chorioretinitis and ampiginous.Due to the diffuse distributions of the active lesions, theacute clinical course and the HLA-A3/C7 association, we believeit to be distinct from these other white dot syndromes. We callthis entity panretinal acute multifocal placoid pigment epitheliopathyor PAMPPE.
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