Synonyms
Definition
Elastic microfibrillopathy as an ocular manifestation of a systemic disorder is characterized by the deposition of whitish material on and within the structures of the anterior eye segment and is frequently associated with severe chronic secondary open-angle glaucoma and cataract.
Histology
The hallmark of PEX syndrome is a pathologic production and accumulation of an abnormal fibrillar extracellular material in the anterior segment tissues. The characteristic PEX fibrils appear to be multifocally produced by various intraocular cell types including the preequatorial lens epithelium, nonpigmented ciliary epithelium, trabecular endothelium, corneal endothelium, vascular endothelial cells, and virtually all cell types of the iris (Naumann et al. 1998). Accordingly, clinical-histopathologic correlations focus on the involvement of the lens, the zonular apparatus, the ciliary body, the iris, the trabecular meshwork, and the cornea.
Immunohistochemistry...
References
Naumann GOH, Schlötzer-Schrehardt U, Küchle M (1998) Pseudoexfoliation syndrome for the comprehensive ophthalmologist: intraocular and systemic manifestations historical image. Ophthalmology 105(6):951–968
Schlötzer-Schrehardt U (2009) Molecular pathology of pseudoexfoliation syndrome/glaucoma – new insights from LOXL1 gene associations. Exp Eye Res 88(4):776–785
Schlötzer-Schrehardt U, Naumann GOH (2006) Ocular and systemic pseudoexfoliation syndrome. Am J Ophthalmol 141(5):921–937
Yanoff M, Duker JS (2009) Ophthalmology 3rd edition expert consult premium edition: enhanced online features and print. Mosby/Elsevier, London, pp 1172–1174
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag Berlin Heidelberg
About this entry
Cite this entry
Kook, D., Kohnen, T. (2015). Pseudoexfoliation Syndrome. In: Schmidt-Erfurth, U., Kohnen, T. (eds) Encyclopedia of Ophthalmology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-35951-4_493-2
Download citation
DOI: https://doi.org/10.1007/978-3-642-35951-4_493-2
Received:
Accepted:
Published:
Publisher Name: Springer, Berlin, Heidelberg
Online ISBN: 978-3-642-35951-4
eBook Packages: Springer Reference MedicineReference Module Medicine