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Updated: Sep 8 2017

Bullous Pemphigoid


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  • Snapshot
    • A 75-year-old woman living in a nursing home presents with a 1-month history of severe pruritus. She reports having unrelenting pruritis with a pink rash over her trunk and extremities. Though she tried multiple lotions, the pruritus does not improve. Approximately 2 days ago, she also noticed some blisters on her body. She has never had rashes like this before. On physical exam, she has several 1-3 cm tense bullae on her abdomen and upper arms. Nikolsky sign is negative. A skin biopsy is taken and sent for immunofluorescence.
  • Introduction
    • Clinical definition
      • bullous pemphigoid (BP) is an autoimmune blistering disorder characterized by bullae (> 1 cm large, fluid-containing blister) and severe pruritus
    • Epidemiology
      • demographics
        • more common in those > 70 years of age
    • 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
    • Associated conditions
      • drug use
        • loop diuretics
        • metformin
        • neuroleptics
      • neurologic conditions
        • multiple sclerosis
        • dementia
        • Parkinson disease
    • Prognosis
      • can resolve spontaneously
      • often recurs
  • 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
        • typically symmetrically distributed on trunks and extremities
      • 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
    • Labs
      • autoantibodies in serum detected with enzyme-linked immunosorbent assay (ELISA)
        • for monitoring disease activity
        • anti-BP180 antibodies
        • anti-BP230 antibodies
    • Histology
      • subepidermal blister with eosinophils
      • direct immunofluorescence
        • linear pattern of IgG and C3 deposition along the epidermal-dermal junction
  • 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
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