Encyclopedia of Ophthalmology

Living Edition
| Editors: Ursula Schmidt-Erfurth, Thomas Kohnen

Pemphigoid, Cicatricial

  • Joshua Zaffos
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-35951-4_917-1



Mucous membrane pemphigoid is a chronic inflammatory disease that affects mucous membranes of the eyes, gastrointestinal system, skin, and genitals. When the eyes are involved, the term ocular cicatricial pemphigoid (OCP) is used.


OCP is thought to represent a cytotoxic, type II hypersensitivity where autoantibodies are directed against cell surface antigens such as bullous pemphigoid antigen II (BP 180), located in the epithelial basement membrane zone (Weisenthal 2013). When these autoantigens are activated in the eye, resultant inflammation and breakdown of the conjunctival surface occur: a process termed cicatrization. IL-1, TNF-alpha, and macrophage-colony stimulating factor are all found to be overexpressed in OCP (Weisenthal 2013). When severe or left untreated, OCP can result in ocular surface scarring and vision loss.

Clinical Presentation

Ocular Manifestations

  • Symptoms:


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  4. Weisenthal RW (2013) Basic and clinical science course. External disease and cornea, pp 198–203Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Department of OphthalmologyKrieger Eye Institute, Sinai Hospital of BaltimoreBaltimoreUSA