From the Guidelines
Bullous Pemphigoid (BP) treatment should prioritize superpotent topical corticosteroids as the first-line treatment for localized and generalized disease, with oral corticosteroids as an alternative for severe cases, based on the most recent and highest quality evidence from 2015 1. The treatment approach for BP depends on the disease severity and extent. For mild localized disease,
- high-potency topical corticosteroids like clobetasol 0.05% cream applied twice daily for 2-4 weeks is recommended as the first-line treatment.
- In cases of localized disease, topical corticosteroids can be applied directly to the lesions. For more extensive or severe disease,
- oral prednisone at 0.5-1 mg/kg/day is recommended, with gradual tapering once disease control is achieved, as supported by earlier studies 1. Steroid-sparing agents are often added for long-term management, including
- azathioprine (1-2.5 mg/kg/day),
- mycophenolate mofetil (1-2 g/day),
- dapsone (50-200 mg/day), as suggested by the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology 1. For refractory cases,
- rituximab (1000 mg IV on days 0 and 14) or
- intravenous immunoglobulin (2 g/kg over 3-5 days) may be used. Supportive care includes
- gentle skin cleansing,
- avoiding trauma to blisters, and
- applying petroleum jelly to erosions. Treatment typically continues for 6-18 months with regular monitoring for medication side effects and disease activity, aiming to suppress the autoimmune response targeting hemidesmosomes in the basement membrane zone, which causes the characteristic subepidermal blistering in pemphigoid. Key considerations in treatment decisions should prioritize minimizing morbidity, mortality, and improving quality of life, as emphasized by the need for careful management of corticosteroid treatment side effects, which were significantly higher in the prednisone 1 mg/kg group, as noted in earlier studies 1.
From the Research
Treatment Options for Bullous Pemphigoid (BP)
- Topical corticosteroids are a treatment option for BP, with studies showing that class I topical corticosteroids can be effective in treating mild forms of the disease 2, 3.
- Whole body application of a potent topical corticosteroid, such as clobetasol propionate cream, can be effective and safe in the induction phase of treatment for both mild and severe BP 3.
- A comparison of two regimens of topical corticosteroids found that a mild regimen using lower doses of topical CS and a shorter duration can improve the outcome of BP patients, particularly those with moderate disease 4.
- Systemic corticosteroids are also a common treatment for BP, but they can have significant side effects, and steroid-sparing agents such as azathioprine, mycophenolate mofetil, and rituximab may be used in combination with CS to reduce these side effects 5, 6.
Specific Treatment Regimens
- A study found that treatment with clobetasol propionate 0.05% cream at an initial dose of 12 mg/m2/day, progressively tapered over months, resulted in remission in 35% of patients and healing in 35% of patients 2.
- Another study found that whole body application of clobetasol propionate cream at doses ranging from 20-40 g/day resulted in disease control in 90% of patients with mild BP and 73.5% of patients with severe BP 3.
- A mild regimen of topical corticosteroids, using lower doses of CS and a shorter duration, was found to be non-inferior to a standard regimen in terms of BP control, and was associated with a reduced risk of death or life-threatening adverse events in patients with moderate BP 4.
Comparison of Guidelines
- A review of guidelines for the management of pemphigus found that there are differences in the recommended initial dose, tapering schedule, and management of relapse for systemic corticosteroids, as well as in the use of rituximab and other immunosuppressive therapies 6.
- The review highlighted the need for standardized guidelines and further research to optimize the treatment of pemphigus and BP.