Original ArticleEfficacy and safety of azathioprine and dapsone as an adjuvant in the treatment of bullous pemphigoid
Introduction
Bullous pemphigoid (BP) is an autoimmune disease, characterised by subepidermal blistering. The disease usually presents in elderly patients. Bullous pemphigoid rarely affects the mucous membranes and is associated with substantial morbidity. The disease express autoantibodies directed against cutaneous autoantigens, BP230 and BP180, called antigen 1 and 2 of BP respectively, both located in the hemidesmosome and anchoring filaments.1
The treatment of choice are systemic steroids, like prednisone, at the dose of 0.5–0.75 mg/kg/day,1, 2 however, class I topical steroids are preferred in localised forms.3 Systemic steroids improve patients’ survival in BP, but also increase the risk of death and life-threatening adverse events.4 Other immunosupresant drugs, called adjuvants, have been widely used to treat autoimmune diseases (e.g. Pemphigus vulgaris) to achieve a corticosteroid-sparing effect.3, 4 The most frequently used agent is azathioprine, while dapsone is another effective option. Therefore we decided to conduct a retrospective study to evaluate the effectiveness and safety of azathioprine and dapsone in BP.
Section snippets
Material and methods
We selected the records of the patients with BP, diagnosed by clinical (lesions suggestive of BP), histological (subepidermal blister) and/or immunological criteria (linear deposition of IgG and/or C3 at the dermoepidermal junction), admitted at the Department of Dermatology, General Hospital of Mexico, in the period from January 2006 to January 2010. All patients had received prednisone 0.5–0.75 mg/kg/day in combination with either azathioprine (group 1, 2–3 mg/kg/day) or dapsone (group 2, 100
Statistical analysis
We perform a Wilcoxon test to evaluate the primary outcome measure. Dichotomous and ordered categorical data were analysed with the Fisher exact and the Mann Whitney test respectively. The analysis was conducted using the statistical program SPSS (version 12 for Windows, Chicago, Ill., USA).
Baseline
We selected 15 records of patients with BP, eight (53%) with azathioprine and seven (47%) with dapsone (demographic and clinical data of the sample are presented in Table 1). All patients represented newly diagnosis BP cases. The mean age was 65.36 ± 6.69 years. The approximate body surface affected (BSA) was 26.2 ± 4.85% (the calculation was made based on the rule of nine). In group 1 (azathioprine), the mean age was 66.4 ± 7.83 years, with BSA of 24.65 ± 6.13%, while in the dapsone group, the average
Discussion
Bullous pemphigoid is a chronic, autoimmune, bullous disease, most commonly seen in the elderly.5, 6 The incidence does not vary among male and female.7 Bullous pemphigoid represents one of the most common subepidermal autoimmune blistering diseases. Patients generally exhibit disseminated lesions consisting in tense blisters, variable in number and size, accompanied by moderate to severe pruritus, often with erythematous or urticarial lesions that may precede the blister, and subsequently
Conflicts of interest
The authors have no conflict of interest to declare.
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Updated French guidelines for the therapeutic management of bullous pemphigoid
2022, Annales de Dermatologie et de VenereologieCitation Excerpt :Patients with recalcitrant forms of BP who do not achieve control of disease activity and those with relapsing forms of BP are usually treated with immunosuppressive drugs, mainly methotrexate [3–6] or mycophenolate mofetil [7–9]. Since the last version of the French BP Guidelines [10], new therapeutic options have been tested in open trials or RCT, in particular methotrexate, biologics (rituximab and omalizumab), and imunomodulators (dapsone and doxycycline), whose indications remain unclear [11–27]. In addition, despite the lack of strong evidence in the literature, the 2015 Guidelines of the European Academy of Dermatology and Venereology (EADV)/European Dermatology Forum (EDF) proposed a mean dose of prednisone/prednisolone of 0.5 mg/kg/day as an alternative to topical corticosteroids [28].
Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy
2021, Journal of the American Academy of DermatologyCitation Excerpt :An alternative or additional second-line, steroid-sparing agent may be needed for patients who have BP that is resistant to tCS, sCS, or doxycycline with niacinamide; who are unable to taper off sCS without a flare up of the disease; or who are experiencing substantial adverse effects from sCS. These second-line steroid-sparing agents include dapsone, sulfapyridine, or oral immunosuppressive agents, such as methotrexate, azathioprine, or mycophenolate mofetil.14,21-27 In addition to its favorable effect on disease activity in BP, dapsone is also an excellent agent for Pneumocystis jirovecii pneumonia prophylaxis.28
Azathioprine: its uses in dermatology
2020, Anais Brasileiros de DermatologiaCitation Excerpt :At a four-year follow-up, 44% of patients achieve remission of the disease.1 It halts disease progression and promotes re-epithelialization as soon as eight weeks of starting treatment.21 MMF is less hepatotoxic, but five times as costly.
Bullous pemphigoid: Associations and management guidelines: Facts and controversies
2013, Clinics in DermatologyCommentary for the Elderly in the Pandemic Era
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