Evidence-Based Management of Bullous Pemphigoid

Bullous pemphigoid (BP) is the most common autoimmune subepidermal bullous disease typically affecting the elderly. Although different therapeutic regimens have been proposed, a review of the evidence is needed to aid clinicians in their decision making and management. Systemic therapies such as corticosteroids and adjuvants are effective in BP but are plagued with adverse effects, and potent topical steroids are an alternative treatment. This article reviews the evidence supporting different therapeutic options in the management of BP.

Bullous pemphigoid (BP) is the most common autoimmune bullous disease, preferentially affecting the elderly. It is characterized by generalized pruritus with subsequent subepidermal bullous formation as well as the detection of autoantibodies against the BP180 (BPAG2, type XVIII collagen) and BP230 (BPAg1-e) antigens. Multiple treatments have been used, though evidence supporting their use is deficient because of a paucity of adequate clinical trials. This shortcoming is partly due to the low prevalence and heterogeneous nature of the disease, and differing study designs with small numbers of patients.

Elderly patients are more likely to have multiple comorbidities and be susceptible to changes in medications than other populations. As a result, adverse effects and potential drug interactions should be considered when treating BP. It is therefore important to provide a review of the evidence supporting the various treatments for BP, so that clinicians can select the best and most efficient medication for their patients. Each treatment regimen must be individualized according to the severity of disease, comorbidities, and patients’ expectations. Furthermore, the physician’s personal experience and drug availability also affect the final choice. Clinical manifestations, epidemiology, and pathogenesis have been reviewed in the previous issue of Dermatologic Clinics . This article focuses on the adaptation of the evidence-based trials in BP to a practical system of managing BP. The authors consider the treatment approach under 3 headings: (1) the context, (2) the treatments, and (3) the morbidity and mortality.

The context of the treatment

The average age of presentation in BP is usually between 75 and 85 years, with many comorbidities, and hence there is a need to be cautious with treatments. BP patients are often either in nursing homes or are already dependent on relatives for care. Three independent case-control studies have shown that there is a high risk of prior neurologic problem 5 to 10 years before the onset of BP. Hence, when treating patients with BP, consideration should be given to 3 important factors that may increase the morbidity and/or mortality of the treatment: (1) patient age, (2) underlying diagnosis such as diabetes mellitus, hypertension, or cardiovascular disease, and (3) the multitude of side effects associated with the use of high doses of systemic corticosteroids. The drugs given to this population need to be tempered with the understanding that they could interfere with other medical problems and will require the assistance of third parties to administer it in most cases. The first consideration is to do no more good than harm to this vulnerable group of patients, no matter how effective a particular treatment may appear in trials perhaps involving healthier patients.

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