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Ocular cicatricial pemphigoid

INTRODUCTION

Mucous membrane pemphigoid is a heterogeneous group of chronic inflammatory blistering diseases that affect oral, ocular, pharyngeal, laryngeal, genital, or anal mucosa. The characteristic pathological feature that unites these disorders is the presence of linear deposits of IgG, IgA, or C3 in the epithelial basement membrane zone. The term ocular cicatricial pemphigoid (OCP) refers to mucous membrane pemphigoid that clinically presents as a chronic cicatrizing (scarring) conjunctivitis. Involvement of other mucosal sites and nonmucosal skin may also occur in OCP.

Left untreated, OCP eventually results in severe conjunctival scarring and visual loss. Thus, early diagnosis and prompt initiation of therapy are essential for optimal management. The treatment of OCP involves the suppression of conjunctival inflammation via the use of systemic immunomodulatory drugs. Conscientious ocular care to minimize the secondary consequences of chronic inflammation also is important.

The pathogenesis, clinical manifestations, diagnosis, and treatment of OCP will be reviewed here. Other forms of pemphigoid are discussed separately. (See "Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid".)

EPIDEMIOLOGY

The incidence of OCP is estimated to be between 1 in 8000 and 1 in 46,000 ophthalmic patients [1-4]. No geographical or racial predilection has been detected.

OCP is primarily a disease of older adults, with an average age of diagnosis between the ages of 60 and 70 years [5-7]. However, due to the nonspecific conjunctival inflammation that characterizes early stage OCP, in some patients the disease may be present for years prior to recognition of the diagnosis.

                          

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Literature review current through: Nov 2014. | This topic last updated: Dec 6, 2013.
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