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Updated: Dec 17 2021

Bullous Pemphigoid

Images junction.jpg pemphigoid.jpg
  • Snapshot
    • A 80-year-old man presents with a 2-week history of severe pruritus and eczema. His past medical history includes atopic dermatitis, thyroid disease, and multiple sclerosis. On physical exam, he has dozens of 1-2 cm tense bullae and several erosions over his upper and lower extremities. His oral mucosa is not involved. Nikolsky sign is negative. A skin biopsy is taken and anti-BP180 is detected in his blood. A potent topical steroid is prescribed.
  • Introduction
    • Clinical definition
      • bullous pemphigoid (BP) is an autoimmune blistering disorder characterized by bullae (> 1 cm large, fluid-containing blister) and severe pruritus
    • Associated conditions
      • drug use
        • loop diuretics
        • metformin
        • neuroleptics
      • neurologic conditions
        • multiple sclerosis
        • dementia
        • Parkinson disease
  • Epidemiology
    • Demographics
      • more common in those > 70 years of age
      • rarely in infants
    • Pathogenesis
      • auto-antibodies (IgG) against hemidesmosomes in the epidermal-dermal junction
        • antibodies are below the epidermis
      • main autoantigens are BP180 and BP230
      • this activates complement and inflammatory reaction which cause epidermal-dermal splitting
  • Presentation
    • Symptoms
      • severe pruritus
      • may have history of eczematous or urticarial lesions before bullae formation
    • Physical exam
      • tense bullae with clear exudate
        • may be hemorrhagic
        • does not easily rupture
        • in adults
          • typically symmetrically distributed on trunks and extremities
        • in infants
          • palms and soles more commonly affected
      • spares mucous membranes
      • negative Nikolsky sign
        • cannot cause separation, blister formation, or blister extension with blunt pressure or lateral traction (dragging finger on skin)
      • may have vesicles
  • Studies
    • Based primarily on clinical presentation and observation
    • Skin biopsy
      • shows subepidermal blisters which are often with eosinophil-rich infiltrates
    • Immunofluorescence
      • shows a linear band of immunoglobulins targeting hemidesmosomes and complement at epidermal-dermal junction
    • Complete blood count (CBC)
      • may show eosinophilia
  • Differential
    • Pemphigus vulgaris
      • more severe than BP
      • affects mucous membranes
      • positive Nikolsky sign
  • Treatment
    • Conservative
      • discontinue new medications
        • indications
          • if BP is suspected to be a drug reaction
      • wound care
        • indications
          • all patients
        • antiseptic care for erosions
    • Medical
      • topical corticosteroids
        • indications
          • first-line treatment
          • often used with systemic treatments
        • drugs
          • clobetasol
      • prednisone
        • indications
          • for widespread disease or if topical steroids are not feasible
      • steroid-sparing immunosuppressant
        • indications
          • for those contraindicated to steroids
        • drugs
          • azathioprine
  • Complications
    • Skin and soft tissue bacterial infection of open lesions
  • Prognosis
    • Can resolve spontaneously
    • Often recurs
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