Prevalence and clinical features of thai patients with bullous pemphigoid

Abstract

Background: Bullous pemphigoid (BP) is a rare, subepidermal autoimmune blistering disease. Studies from different regions show discrepancies in clinical features and courses. Objectives: To reveal clinical characteristics, investigations and clinical outcomes of Thai patients with BP and to evaluate the association of BP with malignancy, diabetes mellitus and neurologic diseases. Methods: Patients diagnosed as BP who had visited the autoimmune skin clinic at Siriraj Hospital between 1991 and 2009 were retrospectively studied. Results: Fifty-eight patients were enrolled. Mean age of onset was 69.3 years. The female to male ratio was 2.7:1. Fifteen percent of the patients had mucosal involvement and 38.9% showed peripheral blood eosinophilia. The sensitivity of the direct and indirect immunofluorescence test in the diagnosis of BP was 95.7% and 73.5%, respectively. The frequency of diabetes mellitus in BP patients was significantly higher than that in the general population (p < 0.001). BP patients had a significantly higher chance of having neurologic diseases compared with other autoimmune vesiculobullous disease patients (adjusted odd ratios 4, 95% confidence interval 1.2-13.3). Disease control was achieved in 89.7% of the patients. One-year and three-year remission rate was 6.4% and 66.3%, respectively. Conclusions: BP usually occurred in the seventh and eighth decade of life and affected females more than males. BP is associated with diabetes mellitus and neurologic diseases. Corticosteroids are the mainstay of the treatment. Two-thirds of patients are likely to be in remission within three years.