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Cause And Symptoms

Complications

Diagnosis And Treatment

Overview

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References

bullous-pemphigoid

Bullous Pemphigoid

<h2>Bullous pemphigoid: A chronic disease with high clinical burden, mortality rates, and unmet needs1–6</h2>
<h3>Defining bullous pemphigoid</h3>
<h4>Bullous pemphigoid is a chronic relapsing autoimmune skin disease, predominantly in those ≥60 years of age, characterized by intense itch, inflammatory lesions, and blister formation1, 6–11</h4>
Bullous pemphigoid at a glance…

Diagnosis of bullous pemphigoid can be delayed:&nbsp;The heterogeneous clinical presentation of bullous pemphigoid, in addition to the complexity of diagnostic tests, can lead to underdiagnosis7,12–15

Bullous pemphigoid can persist for months to years:&nbsp;Bullous pemphigoid has a mean duration of 105 months and a high relapse rate,1,6,16&nbsp;with up to&nbsp;53% of patients relapsing within 6 months of disease remission1

Intense itch is almost invariably present in patients with bullous pemphigoid:&nbsp;85% of patients with bullous pemphigoid suffer daily from itch11

Bullous pemphigoid erosions or blisters are often associated with pain:&nbsp;53% of patients with bullous pemphigoid report pain and of these, 26% report moderate-to-severe pain17

Type 2 inflammation is a critical driver of bullous pemphigoid pathogenesis:&nbsp;The pathophysiology of bullous pemphigoid involves autoimmunity to proteins BP180 and BP230 (components of adhesion complexes in the epidermis) and type 2 inflammation18–21

<h3>A typical patient with bullous pemphigoid:</h3>

<h3>The incidence of bullous pemphigoid varies globally,15, 31–37&nbsp;with the reported worldwide incidence being 0.21–7.63 patients per 100,00038</h3>
The incidence of bullous pemphigoid has been rising over the past two decades, in part due to better recognition of clinical presentations of the disease, increased life expectancy of the general population and the increased use of certain drug triggers, such as:15,31,32,39–41
<ul>
 <li>Dipeptidyl peptidase-4 (DPP-4) inhibitors (gliptins)&nbsp;</li>
 <li>Programmed death (PD)-1/programmed death-ligand (PDL)-1 inhibitors (and other immune checkpoint inhibitors)</li>
 <li>Loop diuretics</li>
 <li>Penicillin</li>
</ul>

<h3>What are the clinical presentations of bullous pemphigoid?</h3>
Bullous pemphigoid is characterized by intense itch and painful polymorphous lesions7,8,11,17,42–44
Bullous pemphigoid can present with or without bullous lesions7,15, 42–45
<ul>
 <li>Bullous presentation (~80%): Can consist of localized or generalized skin blisters arising from erythematous inflamed or normal-appearing skin7,8,42–44</li>
 <li>Non-bullous presentation (~20%): Can consist of urticarial, papular, eczematous lesions, and excoriations may be observed in the early phase of the disease or as the only manifestation of disease in certain patients7,8,15, 42–44</li>
</ul>

<h3>The non-bullous presentation of bullous pemphigoid can be confused with other dermatological conditions, such as atopic dermatitis or urticaria, adding to the burden of underdiagnosis; therefore, accurate identification is important7,13,46</h3>
<h3>How is bullous pemphigoid diagnosed?&nbsp;</h3>
Clinical assessment and obtaining patient history, in addition to techniques such as histopathology, immunofluorescence and/or ELISA are essential for bullous pemphigoid diagnosis; however, access to these resources vary widely by country and region12–14,47
Diagnosis of bullous pemphigoid12,13,42,47,48

<h3>In addition to the heterogenous clinical presentation of bullous pemphigoid, the complexity of diagnostic tests can contribute to underdiagnosis and diagnostic delays of up to 22 months7,12–16</h3>

Bullous pemphigoid in skin of color

Learn about the key challenges to consider when treating bullous pemphigoid

The challenges of managing bullous pemphigoid

Learn about the significant burden experienced by patients with bullous pemphigoid

Morbidity and mortality in bullous pemphigoid

Learn about autoimmunity and the role of type 2 inflammation in the pathophysiology of bullous pemphigoid

What are the contributing factors in bullous pemphigoid?

Learn more about bullous pemphigoid treatment approaches and the current unmet needs

Current treatment approaches and unmet needs in bullous pemphigoid

The heterogeneous presentation of bullous pemphigoid may shed light on why patients are underdiagnosed. Read the article to learn more

What is Bullous Pemphigoid

BULLOUS-PEMPHIGOID

DERMATOLOGY

<h2>Mortality can be more than three times higher in patients with bullous pemphigoid compared with the age-matched general population1</h2>

<h3>The mortality of bullous pemphigoid</h3>
<h4>The 1-year mortality rate ranges from&nbsp;20 to 40%&nbsp;in patients with bullous pemphigoid1–5</h4>
<h4>In a systematic review and meta-analysis of observational studies, a 3.4-fold increased mortality was observed among patients with bullous pemphigoid compared with the age-matched general population1</h4>
A Kaplan-Meier curve depicting survival in patients with bullous pemphigoid vs general population. Patients with bullous pemphigoid have a 3.4-fold increased mortality (N=287)a 1

Figure adapted from Kridin K, et al. Acta Derm Venereol. 2019
aRetrospective cohort study comparing mortality in patients with bullous pemphigoid (N=287) diagnosed between 2000 and 2015 in Israel versus age- and sex-matched controls.1

<h3>Prognostic factors associated with mortality in bullous pemphigoid6–11</h3>
Various factors are associated with poor disease outcomes in bullous pemphigoid:

Prognostic variables for&nbsp;mortality in bullous pemphigoid&nbsp;can include:
<ul>
 <li>Higher dosage of systemic corticosteroids/immunosuppressive drugs6</li>
 <li>Older age at onset and poor performance scale (Karnofsky)&nbsp;6–9</li>
 <li>Low serum albumin levels6</li>
 <li>Multimorbidity and certain comorbidities (e.g. neurological, cardiac, and renal comorbidities)8,10,11</li>
</ul>

High serum anti-BP180 IgG levels are&nbsp;associated with increased 1-year mortality rates&nbsp;in bullous pemphigoid12,13
Additionally,&nbsp;autoantibody profiles may help predict bullous pemphigoid disease course and occurrence of relapse12,14,15

<h3>The impact of bullous pemphigoid on patients’ lives</h3>
<h4>Bullous pemphigoid often leads to substantial physical discomfort and limitations from severe itch and painful eczematous lesions, blisters, and erosions.16–21 This can result in mental, emotional, and socioeconomic burdens and severely impair patients’ quality of life.19, 22–24</h4>

Learn about bullous pemphigoid and its clinical characteristics

What is bullous pemphigoid?

The challenges of managing bullous pemphigoid

What are the contributing factors in bullous pemphigoid?

Current treatment approaches and unmet needs in bullous pemphigoid

Many factors, including older age and multimorbidity, are associated with mortality in bullous pemphigoid. Read this article to find out more

Patient morbidity and mortality in BP

<h2>Bullous pemphigoid management: The complexities of treating a chronic skin disease in a fragile patient population</h2>

<h3>Bullous pemphigoid can have relapse rates of up to 53%, regardless of treatments1,2</h3>

The relapse rate of bullous pemphigoid is high – up to 53%, and the majority of relapses occur within 6 months of remission1

<h3>Estimated relapse rates of patients with bullous pemphigoid at different timepoints after disease remission or treatment withdrawal, based on a meta-analysis study1</h3>

Figure adapted from Wang Y, et al. Ann Med. 2018.

<h3>The ultimate goal of bullous pemphigoid management is long-term disease control3</h3>
Treatment for bullous pemphigoid should aim to:3–7

<h3>Treatment strategies should account for the distinct challenges of bullous pemphigoid8–12</h3>
As bullous pemphigoid is most prevalent in the elderly, treatment strategies should consider the following key challenges of older patients with bullous pemphigoid8–12

High burden of bullous pemphigoid itself, and comorbidities9,11

Increased risk of adverse events with corticosteroids and other immunosuppressants 8,9,13

Risks associated with polypharmacy12

Age-related changes in drug metabolism and decreased drug tolerance10

<h3>Due to the chronicity of bullous pemphigoid, a safe and effective long-tern management approach is needed to reduce the risk of relapse and achieve disease remission11</h3>

What is bullous pemphigoid?

Morbidity and mortality in bullous pemphigoid

What are the contributing factors in bullous pemphigoid?

Learn more about bullous pemphigoid treatment approaches and the current challenges

Current treatment approaches and challenges in bullous pemphigoid

The management of bullous pemphigoid is challenging and new treatment approaches are needed. Read this article to find out more.

Bullous Pemphigoid

Latest Articles

What is Bullous Pemphigoid

Patient morbidity and mortality in BP

Considerations for bullous pemphigoid management