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Immunohistochemical Analysis of a Vitreous Membrane Removed from a Patient with Incontinentia Pigmenti-Related Retinal Detachment

This is a case history of a 23-year-old woman suffering from incontinentia pigmenti (IP). The patient’s vision in the left eye started to deteriorate due to cataract progression at the age of 22, and by the age of 23, it dropped from 0.9 to 0.04. Ultrasound examination confirmed tractional vitreoret...

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Detalles Bibliográficos
Autores principales: Janáky, Márta, Hári Kovács, András, Jánossy, Ágnes, Török, Dóra, Ivanyi, Béla, Braunitzer, Gábor, Benedek, György
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7158695/
https://www.ncbi.nlm.nih.gov/pubmed/31906444
http://dx.doi.org/10.3390/vision4010005
Descripción
Sumario:This is a case history of a 23-year-old woman suffering from incontinentia pigmenti (IP). The patient’s vision in the left eye started to deteriorate due to cataract progression at the age of 22, and by the age of 23, it dropped from 0.9 to 0.04. Ultrasound examination confirmed tractional vitreoretinal membranes. Vitrectomy was performed, therefore, on her left eye. The histological evaluation of vitreous membrane revealed a complex immunophenotype (positivity for glial fibrillary acidic protein (GFAP), vimentin, S-100, anti-pan cytokeratin antibody (AE/AE3), and smooth muscle-specific actin (SMA) to various extents). The right eye remained unsymptomatic throughout this course. Besides being the first to analyze the tractional vitreoretinal membrane in IP with immunohistochemical methods, this case study points out that extreme cases of asymmetric side involvement in IP do exist, even to the point of one eye being completely unsymptomatic.