Skin-nontumor / Clinical dermatology
Blistering disorders
Bullous pemphigoid

Author: Mowafak Hamodat, M.D., MB.CH.B, MSc., FRCPC (see Authors page)

Revised: 10 June 2016, last major update July 2011

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PubMed Search: Skin bullous pemphigoid [title]

Cite this page: Bullous pemphigoid. PathologyOutlines.com website. http://pathologyoutlines.com/topic/skinnontumorbullouspemphigoid.html. Accessed December 7th, 2016.
Definition / General
  • Common subepidermal, blistering, autoimmune disease of skin due to IgG antibodies to bullous pemphigoid antigens 1 and 2
Epidemiology
Etiology
Diagrams / Tables

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Mechanisms of blister formation

University of Oulu, Finland

Clinical Features
  • 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
  • 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

Micro Description
  • 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
  • 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
  • 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