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Skin-nontumor / Clinical Dermatology

Blistering disorders

Bullous pemphigoid


Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC (see Reviewers page)
Revised: 26 August 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Description
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● Common subepidermal, blistering, autoimmune disease of skin due to IgG antibodies to bullous pemphigoid antigens 1 and 2

Etiology
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● Antibodies against hemidesmosomal proteins BP230 (BPG1) and BP180 (BPG2, type XVII collagen) (J Invest Dermatol 2009;129:822)
● Rarely occurs due to trauma (Indian J Dermatol Venereol Leprol 2009;75:617) or drugs (etanercept - a TNF-alpha blocker, Dermatology 2009;219:357; Efalizumab - anti-CD11a, Dermatology 2009;219:89)

Epidemiology
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● Incidence of 10-15 new cases per million people per year (J Dtsch Dermatol Ges 2009;7:434, Br J Dermatol 2009;161:861), but depends on age of population since incidence significantly increases after age 70 years

Drawings
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Mechanisms of blister formation

   

University of Oulu, Finland

Clinical
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● Large tense blisters on flexor surfaces, trunk, intertriginous regions and mucosa
● Bullae don't rupture easily, and heal without scarring
● Self-limiting disease that usually completely clears up; treatment usually needed for several years, but dosage can be reduced after several months
● May flare up after years without symptoms
● A cluster of jewels appearance of new blister arising at the edge of resolving lesions as seen in linear IgA disease may, however, occasionally be a feature

Treatment
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● Goal is to decrease blister formation, promote healing of blisters / erosions
● Must determine minimal medication dosages to control disease, while individualizing therapy for each patient based on other disorders present
● Medications include steroids (topical or systemic), tetracycline and immunosuppressives (eMedicine)

Clinical images
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Subepidermal nonacantholytic blisters

Occurring after a burn

Post-surgical

Various images

Micro description
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● Subepidermal nonacantholytic unilocular blisters with festooning (“suspended in a loop between two points”) of dermal papillae, mild dermal infiltrate including eosinophils, no microabscesses
● Early erythematous lesion shows upper dermal edema, perivascular lymphohistiocytic infiltrate, accompanied by conspicuous eosinophils
● Mild interface changes can be seen in early or prodromal lesions
● In established lesion, the changes are mostly of an inflammatory cell rich variant
● Eosinophilic microabscesses and rarely neutrophilic microabscess are seen
● Cell poor (non-inflammatory) features are seen in biopsies taken from inflamed lesion

Micro images
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Eosinophilic infiltrate

               
Subepidermal bullae

   

Linear deposition of IgG in direct immunofluorescence

Indirect immunofluorescence study on salt-split normal human skin substrate with the serum from a patient with bullous pemphigoid detects IgG serum autoantibodies that bind to the epidermal (roof) side of the skin basement membrane

Other images: #1; #2; #3; #4; #5; #6; #7; #8; #9
Type IV collagen on blister floor #1; #2

Positive stains
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● Linear IgG and C3 antibodies to hemidesmosomes at lamina lucida of basement membrane (versus granular pattern for discoid lupus), occasional IgA and IgM
● 70% have circulating antibodies that bind to basement membrane of normal skin or mucous membranes
● Antibodies bind to epidermal side in NaCl split skin test

Differential diagnosis
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Anti-epiligrin cicatricial pemphigoid: usually affects mucus membrane, reactivity on dermal side in NaCl split skin test
Bullous lupus erythematosus: fulfills criteria for SLE, including positive lupus serology (antinuclear antibodies); reactivity on dermal side in NaCl split skin
Dermatitis herpetiformis: papillary neutrophilic microabscesses, basal cell vacuoles, granular IgA pattern in dermal papillae by direct immunofluoresence, no circulating antibodies
Epidermolysis bullosa acquisita: reactivity on dermal side in NaCl split skin

End of Skin-nontumor / Clinical Dermatology > Blistering disorders > Bullous pemphigoid


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