One year follow-up of patients with bullous pemphigoid

Background. - Since high doses of superpotent topical corticosteroids (CS) have been shown to improve the survival of bullous pemphigoid (BP) patients and to be more effective than oral CS, few studies have described the long-term course of the disease of these patients. We report the course of BP in the Limoges region of France based on patients diagnosed successively between 2002 and 2004. Patients and methods. - This was a retrospective, single-centre study of 54 BP patients diagnosed with BP, conducted by the Department of Dermatology of the Limoges University Hospital over a 3-year period. We investigated epidemiological data, initial symptoms, type of treatment received and outcome at six and 12 months. The two primary end-points were disease control at month 12 and the combined outcome of deaths and relapse over the one-year follow-up period. We calculated the number of deaths, patients lost to follow-up, relapses, and remission at months 6 and 12. Results. - Between January 2002 and December 2004, 54 new BP patients were diagnosed in the Limoges Department of Dermatology: 18 male and 36 female, median age 84 years. Forty patients were treated with superpotent topical CS, 14 required adjuvant immunosuppressant treatment (oral CS, methotrexate, dapsone, mycophenolate mofetit, leflunomide). Over the first 6 months, 28 patients could not be further investigated due to death (n = 20) or loss to follow-up (n = 8). In the 26 patients evaluable for treatment efficacy at month 6, control of the disease was achieved in 17 patients, and nine relapses were seen. The one-year Kaplan-Meier survival rate was 56%. Among the 23 patients evaluable at month 12, control of disease was achieved in 13 patients (complete remission without treatment, n = 2; remission with immunosuppressant treatment, n = 8). Therapeutic failure was concluded for 16 patients, eight of whom presented two relapses during the first year of follow-up. Conclusion. - Despite recent therapeutic recommendations for BP, the prognosis of this disease remains severe. The risk of relapse and mortality rates observed in this study should prompt the development of new therapeutic strategies to supplant superpotent topical CS and reduce the morbidity and mortality associated with the disease. (C) 2009 Published by Elsevier Masson SAS.