Ocular Cicatricial Pemphigoid

Clinical Features

Ocular cicatricial pemphigoid is an autoimmune blistering disease that affects the conjunctiva and multiple mucous membranes. If not treated or treated inappropriately, it can cause blindness. Like pemphigus vulgaris (169610), it is a disease associated with antibodies to components of the skin and mucous membranes. Antibody in pemphigus vulgaris is directed against a protein of the epidermal intercellular cement substance (169615), whereas the antigen involved in OCP has not been definitively characterized. Although both diseases are associated with HLA-DR4 in some patients, in other respects the major histocompatibility complex (MHC) associations are quite different. Ahmed et al. (1991) showed that OCP patients have an increased frequency of DQB1*0301 (see HLA-DQB1, 604305).

Miserocchi et al. (2001) reported a case of chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and fatal granulomatosis with polyangiitis (608710), formerly called Wegener granulomatosis. The patient presented with chronic conjunctival inflammation and scarring, peripheral corneal thinning, and Wegener granulomatosis. Conjunctival biopsy disclosed features of active Wegener granulomatosis and ocular cicatricial pemphigoid, which indicated lack of control of both diseases by methotrexate therapy. The authors stressed that ocular involvement in granulomatosis with polyangiitis indicated poor control of the underlying active vasculitis and indicated the need for treatment with cyclophosphamide.