management

Last edited 09/2020 and last reviewed 08/2023

Seek expert advice.

• the aim of treatment is to suppress the clinical signs of bullous pemphigoid (BP) sufficiently to make the disease tolerable to an individual patient (reduction of blister formation, urticarial lesions and pruritus)
• the disease is self-limiting and usually remits within 5 years
• treatments available work via different mechanisms
  • treatments that aim to suppress the inflammatory process, e.g. corticosteroids, antibiotics (e.g. tetracyclines, sulphones) and other anti-inflammatory drugs
  • other immunosuppressive treatments aim to suppress the production of the pathogenic antibodies, e.g. high-dose corticosteroids e.g. prednisolone 30-60 mg per day, azathioprine, methotrexate, cyclophosphamide and cyclosporin
  • plasmapheresis removes pathogenic antibodies and inflammatory mediators
  • immune-modulating treatments include intravenous immunoglobulins
  • there are two approaches to the initial control of the disease, and currently there is insufficient evidence to reject either approach
    • some clinicians favour the use of minimum doses of systemic therapy to control the disease, individualizing treatment and accepting that in the occasional patient more aggressive therapy may be needed
    • other clinicians believe in controlling all patients with high-dose initial therapy. Treatment is tapered once control of the disease has been achieved.
  • during prolonged maintenance treatment the occasional blister is not an indication for increasing the dose of treatment or changing it (1). The treatment should be reduced whenever the disease has been well controlled for a month or more. In this way it is possible to ensure that the patient is not being over-treated
  • omalizumab, an anti-IgE monoclonal drug which has a good safety profile with minimum adverse side effects should be considered when there is a contraindication to the use of intravenous therapies (eg, immunoglobulins, rituximab) or prolonged immunosuppressive treatment (eg, methotrexate, azathioprine) (2)

Reference:

1. Br J Dermatol. 2002 Aug;147(2):214-21.
2. Navarro-Trivino FJ et al. Dramatic improvement of bullous pemphigoid with omalizumab in an elderly patient.European Journal of Hospital Pharmacy Published Online First: 12 September 2020. doi: 10.1136/ejhpharm-2020-002418