Bullous pemphigoid typically occurs in older people and has a distinctive clinical appearance. There are several clinical variants, one of which occurs in childhood.
In the prodromal, non-bullous phase, pruritus of variable intensity may be accompanied by eczematous or urticarial lesions for weeks or months.
In the bullous stage, characteristic, tense vesicles or bullae develop on apparently normal or erythematous skin of the pre-existing eczematous or urticarial eruption. If the blisters burst, the eroded, crusty areas slowly heal to leave post-inflammatory hyperpigmentation.
The lesions are usually symmetrical and favour the flexural aspects of the extremities, lower trunk, and abdomen.
The treatment goal is to decrease or stop blister formation, to promote healing of existing blisters and erosions, and to control the associated pruritus. Topical and systemic corticosteroids, as well as other immunosuppressive and anti-inflammatory agents, are used.
Bullous pemphigoid is a chronic, acquired autoimmune blistering disease characterised by auto-antibodies against hemidesmosomal antigens, resulting in the formation of a sub-epidermal blister.
History and exam
Key diagnostic factors
- presence of risk factors
- tense blisters on normal or erythematous skin
Other diagnostic factors
- erythematous or urticarial plaques
- oral lesions
- age 60 to 90 years
- major histocompatibility complex (MHC) class II allele (DQB1*0301)
- male sex
1st investigations to order
- skin biopsy for histopathological evaluation with light microscopy
- skin biopsy for direct immunofluorescence testing
- indirect immunofluorescence test on serum
Investigations to consider
- ELISA test
- fluorescence overlay antigen mapping (FOAM) technique
localised lesions in children or adults
widespread lesions in adults
widespread lesions in children
- Pemphigus vulgaris
- Epidermolysis bullosa acquisita
- Linear IgA dermatosis
- Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita
- Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)
Karnofsky Performance Status ScoreMore Calculators
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