What is the historical data on bullous pemphigoid?

From the Guidelines

Bullous pemphigoid is a chronic autoimmune skin disease with a significant historical mortality rate of 20-40% in elderly patients, which has been improved with the introduction of various treatments, including systemic corticosteroids, immunosuppressive agents, and targeted therapies. Historically, bullous pemphigoid was first clearly distinguished from pemphigus vulgaris in the 1950s by Walter Lever 1. The disease primarily affects older adults, with the average age of onset being around 70 years. Some key points to consider in the management of bullous pemphigoid include:

• Definitions for bullous pemphigoid, such as control of disease activity, time to control of disease activity, and end of consolidation phase, as outlined in the study by 1
• The use of systemic corticosteroids, such as prednisone, as the mainstay of treatment, often requiring high doses for extended periods, which can cause significant side effects in elderly patients
• The introduction of immunosuppressive agents, such as azathioprine and cyclophosphamide, as steroid-sparing agents in the 1970s and 1980s
• The use of tetracycline antibiotics with nicotinamide as an alternative treatment for milder cases, as well as the more recent introduction of rituximab and other targeted therapies for treatment-resistant cases
• The identification of specific autoantibodies targeting BP180 and BP230 proteins in the basement membrane zone, which has improved diagnostic accuracy through immunofluorescence techniques and ELISA testing, as discussed in the study by 1. Key terms to consider in the management of bullous pemphigoid include:
• Minimal therapy, defined as ≤ 0.1 mg/kg/d of prednisone or 20 g/wk of clobetasol propionate and/or minimal adjuvant or maintenance therapy
• Complete remission on minimal therapy, defined as the absence of new or established lesions or pruritus while receiving minimal therapy for at least 2 months
• Relapse/flare, defined as the appearance of ≥3 new lesions/month or at least one large eczematous lesion or urticarial plaques that do not heal within 1 week, or extension of established lesions or daily pruritus in a patient who has achieved disease control, as outlined in the study by 1.

From the Research

Historical Data on Bullous Pemphigoid

• Bullous pemphigoid (BP) is the most common autoimmune blistering disorder, with a chronically relapsing course 2.
• The disease is caused by autoantibodies against hemidesmosomal adhesion proteins, leading to subepidermal blistering 2.
• Treatment options for BP include topical and systemic corticosteroids, as well as immunosuppressants such as azathioprine and mycophenolate mofetil 3, 2, 4.

Treatment Options

• Topical corticosteroids, such as clobetasol propionate, have been shown to be effective in controlling BP, with fewer systemic side effects than oral corticosteroids 5, 3, 6, 4.
• Systemic corticosteroids, such as prednisone, are also effective, but may be associated with significant side effects, particularly at high doses 3, 6, 4.
• Immunossuppressants, such as azathioprine and mycophenolate mofetil, may be used as adjuvant therapy to reduce the use of corticosteroids 3, 2, 4.

Efficacy and Safety of Treatment Options

• A study published in 2002 found that topical clobetasol propionate was superior to oral prednisone in terms of overall survival and disease control in patients with extensive BP 6.
• A review published in 2011 found that ultrapotent topical corticosteroids, such as clobetasol propionate, were effective treatments for BP, with fewer systemic side effects than oral high-dose corticosteroids 3.
• A Cochrane review published in 2010 found that very potent topical steroids were effective and safe treatments for BP, but their use in extensive disease may be limited by side effects and practical factors 4.