Search

Background: Current treatment of bullous pemphigoid (BP) is based on the long-term use of topical and/or systemic corticosteroids, which are associated with a high rate of adverse events and increased mortality., Objectives: To study the corticosteroid-sparing potential of azathioprine and dapsone., Methods: This was a prospective, multicentre, randomized, nonblinded clinical trial that compared the efficacy and safety of two parallel groups of patients with BP treated with oral methylprednisolone 0·5 mg kg -1 per day in combination with either azathioprine 1·5-2·5 mg kg -1 per day or dapsone 1·5 mg kg -1 per day. Nine German and Austrian departments of dermatology included 54 patients based on clinical lesions, positive direct immunofluorescence (IF) microscopy and detection of serum autoantibodies by indirect IF microscopy, immunoblotting or enzyme-linked immunosorbent assay. The primary end point was the time until complete tapering of methylprednisolone, and the most important secondary end point was the cumulative corticosteroid dose., Results: In eight patients (five azathioprine, three dapsone), methylprednisolone could be discontinued after a median time of 251 days in the azathioprine group and 81 days in the dapsone group. The median cumulative corticosteroid dose was 2·65 g for azathioprine compared with 1·92 g for dapsone (P = 0·06). The median numbers of days when corticosteroids were applied were 148 and 51, respectively (P = 0·24). No significant difference in the number of adverse events was seen between the treatment arms. Four patients (8%) died within the observation period of 12 months., Conclusions: Due to the lower than intended number of patients, the results of the primary and secondary end points were not or only barely significant. Dapsone appeared to have a moderately higher corticosteroid-sparing potential than azathioprine. The combination regimen of either drug with oral methylprednisolone is associated with a relatively low 1-year mortality in this vulnerable patient population., (© 2017 British Association of Dermatologists.)

Background:   It has been previously shown in a relatively small group of patients that a combination of immunoadsorption (IA) and rituximab with daily use of high-dose oral corticosteroids and azathioprine/mycophenolate mofetil may induce a rapid and durable remission in severe, treatment-resistant pemphigus., Objectives: To achieve a more rapid reduction of serum autoantibody levels by a more frequent use of IA in the initial phase of treatment and to reduce the number of severe adverse events of continuous oral corticosteroid therapy by switching to pulsed intravenous applications., Methods: Twenty-three consecutive patients with severe pemphigus were included. IA was performed at initially 3- and later 4-week intervals until lesions healed by 90%; 1000mg rituximab was given at weeks 1 and 3, and intravenous dexamethasone pulses were administered at first every 3weeks and then at increasing intervals in addition to daily azathioprine/mycophenolate mofetil., Results: Along with a fast and durable decline of circulating autoantibody levels, all patients showed improvement of pemphigus lesions within the first weeks of therapy and long-term complete remission was induced in 19 (83%) patients. In the remaining four patients, one (4%) minimal disease and three (13%) partial remissions were observed. Over the long-term follow-up of 11-43 (mean 29) months, six (26%) patients had a recurrence and in two (9%) patients, severe adverse events occurred., Conclusions: This novel protocol treatment induces a fast and long-term remission in severe pemphigus and seems to offer an improved side-effect profile compared with daily use of corticosteroids., (© 2011 The Authors. BJD © 2011 British Association of Dermatologists.)