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Bullous pemphigoid
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Bullous pemphigoid

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Contributors: Jeffrey M. Cohen MD, Belinda Tan MD, PhD, Susan Burgin MD
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Synopsis

This summary discusses adult patients. Bullous pemphigoid of childhood is addressed separately.

Bullous pemphigoid (BP) is a chronic autoimmune subepidermal blistering disease most frequently seen in the elderly. IgG autoantibodies bind to antigens that comprise the hemidesmosome adhesion complex in the basement membrane of the skin (BP180 or BP230). This triggers complement activation and release of inflammatory mediators, resulting in the formation of local or generalized tense bullae. The disease can occur on any body surface, but mucous membrane involvement is rare.

Relapse of BP has been shown to be more likely in individuals with extensive involvement and dementia. BP has been associated with other neurologic disorders. BP has also been associated with other autoimmune diseases such as diabetes mellitus, thyroiditis, dermatomyositis, lupus erythematosus, rheumatoid arthritis, ulcerative colitis, myasthenia gravis, and multiple sclerosis in case reports and case series. A population-based study from Finland strongly suggests an increased risk of BP in patients with dermatitis herpetiformis. Therapeutic radiation or drugs (furosemide, NSAIDs, captopril, penicillamine, gliptins, and some antibiotics) have also been associated with BP. It may also follow certain nonbullous inflammatory skin diseases, such as psoriasis and lichen planus, or vaccination (most often in children).

In patients of Northern European descent, there has been a significant association with the DQB1*0301 allele, whereas patients of Japanese descent have a higher frequency of alleles DRB1*04, DRB1*1101, and DQB1*0302.

The condition is often self-limiting, but it can become chronic over months to years. There is a wide spectrum of clinical severity. The disease can be generalized and severe, or patients may have only a few asymptomatic, localized bullae. There is no ethnic or sex predilection.

In some instances, early BP lesions will appear as pruritic urticarial papules and plaques (known as urticarial BP). The urticarial prebullous phase may vary in length from weeks to many months. Prebullous BP may present with pruritus only or itchy papules or nodules without urticarial plaques. This variant is known as nonbullous BP.

Peripheral eosinophilia is common and is positively correlated with disease severity. Its presence has also been correlated with older age and palmoplantar involvement. Lack of peripheral eosinophilia has been positively correlated with younger age and mucosal disease.

Codes

ICD10CM:
L12.0 – Bullous pemphigoid

SNOMEDCT:
77090002 – Bullous pemphigoid

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Urticarial phase of BP:
Nonbullous BP:

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated: 07/15/2019
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Bullous pemphigoid
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Bullous pemphigoid : Tense bullae, Pruritus, Tense vesicles
Clinical image of Bullous pemphigoid
A close-up of a tense bulla and a vesicle at the edge of a healing eroded plaque.
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