Introduction
Bullous pemphigoid is an autoimmune condition causing sub-epidermal blistering of the skin.
Epidemiology
- Incidence: 1.00 cases per 100,000 person-years
- Peak incidence: 70+ years
- Sex ratio: 1:1
| <1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Pathophysiology
Bullous pemphigoid is caused by the development of antibodies against hemidesmosomal proteins BP180 and BP230.
Clinical features
Bullous pemphigoid is more common in elderly patients. Features include
- itchy, tense blisters typically around flexures
- the blisters usually heal without scarring
- mouth is usually spared
- this is seen as a classic differentiating feature between pemphigoid and pemphigus
- in reality around 10-50% of patients have a degree of mucosal involvement
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| © Image used on license from DermNet NZ | |
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| © Image used on license from DermNet NZ and with the kind permission of Prof Raimo Suhonen | |
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| © Image used on license from DermNet NZ and with the kind permission of Prof Raimo Suhonen | |
Investigations
Skin biopsy
- immunofluorescence shows IgG and C3 at the dermoepidermal junction
Management
Management
- referral to dermatologist for biopsy and confirmation of diagnosis
- oral corticosteroids are the mainstay of treatment
- topical corticosteroids, immunosuppressants and antibiotics are also used