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Bullous Pemphigoid

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Topic updated on 09/08/17 6:18pm

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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Qbank (1 Questions)

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(M1.MK.1) A 50-year-old female presents to her physician with vesicles and tense blisters across her chest, arms, and the back of her shoulders. Physical examination reveals that blistering is not present in her oral mucosa, and the epidermis does not separate upon light stroking of the skin. The patient most likely suffers from a hypersensitivity reaction located: Topic Review Topic

1. Linearly along the epidermal basement membrane
2. In net-like patterns around epidermal cells
3. In granular deposits at the tips of dermal papillae
4. In fat cells beneath the skin
5. In nuclei within epidermal cells

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