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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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