Abstract
Bullous pemphgoid (BP) is an acquired autoimmune subepidermal blisterng disease, which shows various symptoms, including bulla, polymorphic erythema, and pruritus. There is often an eosinophilia and raised serum IgE. The target antigens are hemidesmosomal cytoplasmic BP230 and transmembrane BP180 proteins. In this study, we investigated the correlation among the clinical symptoms, serum IgE, the eosinophil count, indirect immunofluorescence titer, and BP180NC16a scores measured by enzyme-linked immunosorbent assay (ELISA). With clinical improvement, all of these values decrease ; the BP180NC16a scores and the eosinophil count were particularly closely correlated. At the disease relapsed, the BP180NC16a score was the most sensitive indicator. In patients exhibiting high levels of BP180NC16a scores greater than 150 by regular ELISA, we again performed ELISA with appropriate dilutions and obtained true index values that exhibited a better correlation with disease activity. During plasma exchange, we could detect the reduction of the pathogenic anti-BP180NC16a autoantibody by using ELISA. These findings indicate that BP180NC16a ELISA can be valuable not only for the diagnosis of patients with BP but also for monitoring disease activity.
