Bullous Pemphigoid

 

Bullous Pemphigoid

Bullous Pemphigoid (BP) is the most common autoimmune blistering disease, typically affecting the elderly. It is characterized by autoantibodies against hemidesmosomes (specifically BP180 and BP230) at the dermo-epidermal junction.

Clinical Presentation

  • Prodrome: May present with an intensely pruritic, urticarial, or eczematous eruption weeks or months before bullae appear.
  • Bullae: Tense, firm, fluid-filled blisters; often found on flexural surfaces and the trunk.
  • Nikolsky Sign: Negative; because the cleavage is subepidermal, the blister roof is reinforced by the entire epidermis.

Diagnostic Criteria

Test Finding
Histopathology Subepidermal bulla with eosinophilic infiltrate.
Immunofluorescence Linear IgG and C3 deposits along the basement membrane zone.
High-Yield Exam Pearl:

  • Comparison: Unlike Pemphigus Vulgaris, BP usually spares the oral mucosa.
  • Management: High-potency topical corticosteroids (e.g., clobetasol) are often first-line; systemic steroids or immunosuppressants are reserved for extensive disease.