Bullous Pemphigoid
Epidemiology:
Common sites:
- inner thighs
- flexor forearms
- axillae
- groin
- lower abdomen
- oral (10-15%)
- usually after
involvement of cutaneous sites
Gross features:
- tense bullae filled with clear fluid
- do not rupture
easily (unlike pemphigus)
- on normal or erythematous skin
- heal without
scarring if no superimposed infection
- localized or
generalized
- involvement of
mucosal surfaces (but less often than pemphigus)
Histologic features:
- subepidermal blistering (non-acantholytic)
- superficial and
sometimes deep perivascular infiltrate
- lymphocytes, eosinophils, occasional neutrophils
- superficial dermal
edema
- basal layer
vacuolization
Immunophenotype:
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Marker:
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Sensitivity:
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Specificity:
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- linear
zone deposition of immunoglobulin and complement at the dermal-epidermal
junction
Molecular features:
Other features:
- antibodies
directed against attachment placques (hemidesmosomes) between the basal cell and basement membrane
References: