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Introduction
Bullous pemphigoid (BP) is an autoimmune sub-epidermal blistering disease that primarily affects the elderly, typically those between 60 and 80 years of age.1 This condition affects both men and women equally.
In the United Kingdom, the overall prevalence of BP has been reported at 48 cases per 100,000 people.2
Aetiology
The exact aetiology of BP is not fully understood, but it is primarily considered an autoimmune disease. Genetic predisposition plays a role, with certain human leukocyte antigens (HLAs) associated with increased risk.3-4 Â
Environmental triggers, including medications and infections, may contribute to disease onset.3 BP has been linked with neurological conditions such as dementia, Parkinson’s disease, and stroke, suggesting a potential neurological association.3
Pathophysiology
BP is characterised by the production of autoantibodies that target specific proteins in the basement membrane zone, primarily BP antigen 180 (BP180) and BP antigen 230 (BP230). These antigens play a crucial role in the adhesion complexes responsible for maintaining epithelial-stromal adhesion.
When autoantibodies bind to these target antigens, they trigger an inflammatory response that leads to dermal-epidermal separation, resulting in the formation of characteristic sub-epidermal blisters and erosions.1
Risk factors
Risk factors for BP include:1, 3-4
- Advanced age: most cases occur in individuals between 60 and 80 years of age
- Neurological diseases: particularly Parkinson’s disease, dementia and stroke
- Medications: PD1 inhibitor immunotherapies (e.g. nivolumab, pembrolizumab) and DPP4 inhibitors (e.g. linagliptin, vildagliptin)
- Genetic predisposition: association with certain HLA alleles (HLA-DQB1*0301 is associated with an increased risk of BP by 1.64)
Clinical features
History
When taking a history for suspected BP, important features include:
- Itchiness: often severe and may precede blistering by weeks to months
- Skin blisters: tense bullae
- Associated symptoms: burning sensation, pain
- Constitutional symptoms: generally not present, except in severe widespread disease
Important areas of the history
Other important areas of the history to cover include:
- Past medical history: particularly neurological conditions (e.g. dementia, Parkinson’s disease, stroke)
- Medication history: recent changes, especially new medications, including PD1 inhibitors or DPP4 inhibitors
- Family history: given the potential genetic predisposition, other family members may have been affected
Clinical examination
A thorough dermatological examination of the lesions should be performed.
Typical examination findings of BP include:Â
- Erythematous, urticarial plaques: in the pre-bullous stage
- Tense bullae: containing clear or haemorrhagic fluid, which have a flexural, abdominal, and thigh distribution
- Erosions and crusts: in areas where blisters have ruptured
- Mucous membrane involvement: less common than in pemphigus vulgaris
- Secondary infection: can occur within the lesions, particularly in extensive disease
- Scarring: more common in BP due to the deep dermal involvement
Nikolsky sign
This is where slight friction on the skin results in the shearing of the epidermis.
Nikolsky sign is negative in BP.
Differential diagnoses
Other blistering conditions present similarly to BP. Differential diagnoses include:
- Pemphigus vulgaris: flaccid blisters, unlike the tense blisters in BP. Positive Nikolsky sign, which is typically negative in BP
- Linear IgA dermatosis: tense vesicles and bullae in an annular (string of pearls) pattern. Often affects children, while BP typically affects the elderly. Linear IgA deposits at the basement membrane zone are seen on direct immunofluorescence
- Dermatitis herpetiformis: intensely pruritic papulovesicular eruptions on extensor surfaces. Associated with coeliac disease, unlike BP. Granular IgA deposits in dermal papillae are seen on direct immunofluorescence
Table 1. Comparison of bullous pemphigoid and pemphigus vulgaris 1, 3, 5-6
| Bullous pemphigoid | Pemphigus vulgaris | |
| Age group | Elderly | Middle-aged |
| Blister location | Sub-epidermal | Intraepidermal |
| Blister characteristics | Tense, firm | Rupture easily, flaccid |
| Mucosal involvement | Less common | Very common |
| Nikolsky sign | Negative | Positive |
| Autoantigens | BP 180, BP 230 | Desmoglein 1, Desmoglein 3 |
| Histology | Subepidermal blisters with eosinophils | Intraepidermal acantholysis |
| Prognosis | Better | More chronic course, with higher morbidity and mortality |
Pemphigoid and pemphigus
PemphigoiD =Â Deep and pemphiguS =Â Superficial
This shows the fundamental difference between the two conditions, their level of involvement and, therefore, their pathophysiology.
Investigations
The diagnosis of BP typically involves a combination of blood tests and skin biopsy.
Laboratory investigations
Blood tests are crucial in the diagnostic process. Indirect immunofluorescence is used to detect circulating pemphigoid BP 180 and BP 230 autoantibodies.1, 3
Skin biopsy
Two skin biopsy samples are usually required, the first sample is taken from the edge of a blister (lesional) for histological examination. This typically reveals sub-epidermal blisters along with eosinophils at the dermal-epidermal junction.
The second sample is obtained from the skin within 2 cm of the blister (perilesional) for direct immunofluorescence (DIF) testing, which typically shows linear deposition of IgG along the basement membrane and/or C3 deposits.1, 3
Management
The management of BP requires a comprehensive approach tailored to disease severity and individual patient factors.
Supportive management
Supportive care is crucial in PV and includes:
- Correction of fluid and electrolyte imbalances
- Nutritional support
- Pain management
- Wound careÂ
Medical management
Medical management of PV includes:Â 1, 3
- Discontinuing any identified culprit medications
- Topical super-potent corticosteroids: for localised disease
- Systemic corticosteroids (e.g. prednisolone): in more extensive disease
- Anti-inflammatory antibiotics (e.g. doxycycline): may also be beneficial
- Nicotinamide: may be prescribed alongside the above medications
- Immunosuppressive agents: in steroid-resistant disease or where steroids are contraindicated. May include methotrexate, azathioprine, mycophenolate mofetil or rituximab1, 3, 7
- Intravenous immunoglobulin (IVIG): considered if refractory to standard treatment1
Regular follow-up is essential to monitor disease activity, adjust treatment as needed, and manage any potential complications or treatment side effects.Â
Complications
Complications of BP include:
- Secondary infections: bacterial or viral
- Adverse effects from treatment: particularly associated with steroids or immunosuppressive medications
- Impacts on food intake and nutrition, particularly with oral involvement
- Psychological distress
Reviewer
Dr Matt Alexander
Consultant Dermatologist
Editor
Dr Jess Speller
References
- Baigrie D, Nookala V. Bullous Pemphigoid. StatPearls. Treasure Island (FL). 2023. Available from: [LINK].
- Bax CE, Werth VP. The incidence of bullous pemphigoid continues to increase in England. British Journal of Dermatology. 2021. Available from: [LINK].
- Oakley A, Coulson I. DermNet. Bullous pemphigoid. 2024. Available from: [LINK].
- Thibaut D, Witcher R, Barnes B, et al. HLA-DQB1*0301 in bullous pemphigoid and pemphigus vulgaris: A meta-analysis. International Journal of Medical Students. 2023. Available from: [LINK].
- Ingold CJ, Sathe NC, Khan MAB. Pemphigus Vulgaris. StatPearls. Treasure Island (FL). 2024. Available from: [LINK].
- Ngan V, Oakley A, Shakel M. DermNet. Pemphigus vulgaris. 2022. Available from: [LINK]
- Venning VA, Taghipour K, Mohd Mustapa MF, et al. British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012. British Journal of Dermatology. 2021. Available from: [LINK].
Image references
- Figure 1. DermNet. Bullous pemphigoid. License: [CC BY-NC-ND 3.0 NZ].
- Figure 2. DermNet. Bullous pemphigoid. License: [CC BY-NC-ND 3.0 NZ].
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