10-Year Observation of Bullous Pemphigoid Patients

Background and Design: Bullous pemphigoid (BP) is an autoimmune skin disorder characterized by large, tense blisters of the skin. It occurs mainly in the elderly. The one year mortality associated with bullous pemphigoid ranges from 19% to 41%. We aim to analyse the etiologic factors, demographic features, clinical course and response to treatment in patients with BP. Material and Method: Between January 1999 and January 2008, 31 patients with BP were evaluated retrospectively. We assessed duration of the disease, presence of associated symptoms, clinical findings, drug intake, response to treatment, recurrence rates and also extensive laboratory testing and radiologic evaluations on each patient to clarify a possible underlying malignancy. Results: In a 10-year period, 31 patients with BP, female male ratio was 19/12, ranging in age from 59 to 96 (mean 78.8 +/- 8.67 years) were evaluated. Duration of the disease was 16.58 +/- 18.48 months. Direct immunoflourescence of the perilesional skin showed linear deposition of IgG and C3 along the dermoepidermal junction in 24 patients (77.4%). There was no significant relationship between drug usage and bullous pemphigoid. No malignancy was detected. Medium dosage of prednisolone (50-60 mg/day) was the first choice of treatment in 8 patients with a complete response rate in all but two patients died during therapy. Four patients died during hospitalization with unknown reasons before any treatment was started. In 16 cases the initial treatment was prednisolone and azathioprine. The mean follow-up period was 12 months and 11 (35%) cases had relapsed. The longest remission was achieved with prednisolone and azathioprine combination. Conclusion: In conclusion, no association was found between BP and drug usage or malignancy. All patients responded well to treatment. Therapy with prednisone maintained with azathiopyrine significantly reduced the recurrences. However, disease-specific mortality is found to be high and factors for mortality need to be identified. (Turkderm 2010; 44: 61-4)