ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY. STUDY OF 16 CASES EPITELIOPATÍA PIGMENTARIA PLACOIDE POSTERIOR MULTIFOCAL AGUDA. ESTUDIO DE 16 CASOS

Objective: Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare disease with a probable inflammatory component which mostly affects young patients. The aim of our study was to analyse the demographic and clinical features of this disease in a group of 16 patients. Methods: Sixteen patients with APMPPE were included in this study. We analyzed their demographic data (age, sex) and the most relevant clinical findings: visual acuity and retinal disease outcome, association with other systemic diseases and response to treatment. We also collected data from fluorescence angiography, autofluorescence and optical coherence tomography (OCT) in some of the patients. Results: Average age at diagnosis was 26.75 years with no sex predilection. Average final visual acuity (Snellen Scale) in our study was 0.73. Four patients presented with a systemic disease related to the APMPPE. Eleven patients were treated with oral steroids (one patient with steroids and cytotoxic agents) while the remaining 5 patients received no treatment. Conclusions: In our patients, the average age at diagnosis was less than 30 years, with no sex predilection, as previously described by many authors.