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Curr Allergy Asthma Rep. 2005 Jul;5(4):333-8.

Ocular cicatricial pemphigoid: manifestations and management.

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Department of Ophthalmology, St. Vincent's Hospital, Level 10, 438 Victoria Street, Darlinghurst NSW 2010 Australia.


Ocular cicatricial pemphigoid (OCP) is a chronic, progressive cicatrizing inflammatory disease of presumed autoimmune etiology affecting the mucous membranes and skin. It has sight-threatening ocular manifestations and potentially life-threatening extraocular manifestations. The ocular signs include: chronic cicatricial conjunctivitis, progressive conjunctival fibrosis, and corneal epitheliopathy. Systemic immunomodulatory therapy is the treatment of choice for controlling disease activity and limiting progression, given the systemic nature of the disease and the poor efficacy of current local or topical therapies. Systemic cyclophosphamide with short-term adjunctive high-dose prednisolone is the preferred treatment for severe and/or rapidly progressing OCP. Oral low-dose weekly methotrexate is a useful first-line treatment for mild-to-moderate OCP. The management of OCP requires a multidisciplinary approach to optimize the care of these patients.

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