[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. The visual outcome is usually good regardless of the treatment given, although there are cases with a bad visual outcome, especially those with foveal involvement when initially seen.

Epiteliopatía pigmentaria placoide posterior multifocal aguda. Estudio de 16 casos / Acute posterios multifocal placoid pigment epitheliopathy. Study of 16 cases

Objetivo: La Epiteliopatía Pigmentaria Placoide Posterior Multifocal Aguda (EPPPMA) es una enfermedad infrecuente que afecta a individuos jóvenes y de probable etiología inflamatoria. El objetivo del estudio es analizar las principales características clínicoepidemiológicas de esta enfermedad en una serie de pacientes. Métodos: Se presenta un estudio retrospectivo de 16 pacientes diagnosticados de EPPPMA. Se han estudiado sus características demográficas (edad, sexo) y clínicas: evolución de la agudeza visual y del cuadro retiniano, enfermedades sistémicas asociadas y respuesta al tratamiento. Se han recogido datos angiográficos, autofluoresecencia y tomografía de coherencia óptica (OCT) en algunos pacientes. Resultados: La edad media en nuestra serie es de 26,75 años sin preferencias por el sexo. La media de agudeza visual final en nuestra serie ha sido de 0,73. Cuatro pacientes presentaron enfermedad sistémica asociada a la EPPPMA. 11 pacientes recibieron tratamiento con corticoesteroides (un paciente corticoesteroides e inmunosupresores) y cinco no recibieron tratamiento. Conclusiones: En nuestra serie la edad media se encuentra por debajo de los 30 años y no hubo diferencias en cuanto al sexo, siendo estos resultados congruentes con lo descrito en la literatura. El pronóstico visual es bueno independientemente del tratamiento, aunque hay casos de mal pronóstico visual, especialmente aquellos con afectación foveal precoz

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. The visual outcome is usually good regardless of the treatment given, although there are cases with a bad visual outcome, especially those with foveal involvement when initially seen