ORAL PEMPHIGOID - SUBSET OF CICATRICIAL PEMPHIGOID

Objective. Cicatricial pemphigoid (CP) is an autoimmune blistering dis ease characterized by anti-basement membrane zone (BMZ) antibodies wit h a varied heterogeneous clinical spectrum. We sought to characterize a subset of patients with disease limited to the oral cavity. Study De sign. Twenty-nine random patients with vesiculobullous disease limited to the oral cavity were studied. We identified patients by clinical c riteria, the presence of subepidermal/subepithelial bullae on routine histopathologic study, and deposition of IgG, complement, or both on t he BMZ of perilesional tissue by immunopathological studies. Treatment included local and systemic therapies. Patients were monitored for a mean period of 6.7 years (range, 3.5 to 11 years). Results. The female /male ratio was 4.8:1. Patients with limited or minimal disease receiv ed local therapy only. In patients with extensive or severe disease, t he use of dapsone yielded significant clinical improvement. Long-term follow-up showed that patients with severe disease treated with dapson e followed a clinical course similar to that in patients with minimal disease. Conclusions. Oral pemphigoid is a distinct clinical subset of CP. Overall it has a relatively benign course compared with that in p atients with CP involving the oral cavity and other mucosae and the sk in. Patients with minimal disease respond satisfactorily to topical th erapy. Patients with severe and extensive disease benefit from dapsone therapy. In most patients the clinical course is prolonged and treatm ent is required for several months; in our study the mean treatment pe riod was 42 months (range, 24 to 78 months). All the patients in this study went into clinical remission and remained in remission on cessat ion of therapy. No other mucosae or the skin were involved during the follow-up period.