Ocular cicatricial pemphigoid

Abstract

Purpose of review: To review the content and importance of articles on ocular cicatricial pemphigoid (OCP), or mucous membrane pemphigoid (MMP) with relevance to ocular involvement, published from 2005 to 2006.

Recent findings: There is wide antigenic heterogeneity in MMP and OCP and attempts are being made to link this with clinical variations with varying degrees of success. A larger study demonstrates a link between multiple autoantibody reactivity and disease severity. Details of epitope specificity are emerging. A highly sensitive ELISA for laminin 5 has been developed which may provide prognostic information.Clinically, younger OCP patients show more severe ocular disease and are less well controlled by medication. A systematic review shows good evidence only for steroids and cyclophosphamide in OCP. Continued small trials suggest some role for the expensive intravenous immunoglobulin therapy in OCP where conventional treatment fails. Ocular reconstruction surgery has made some progression, and is enhanced by adjunctive medical therapy; osteo-odonto-keratoprosthesis offers some hope of prolonged retention of limited vision in end-stage disease.

Summary: OCP remains a difficult disease to manage well and the progress being made in understanding disease mechanisms is whence true disease-modifying, safe therapy is likely to emerge in the future.