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Updated: Nov 29 2018

Pemphigus Vulgaris

  • Snapshot
    • A 45-year-old woman presents to the physician's office for a painful and blistering rash. She reports having a 1-month history of nonhealing ulcers in her mouth, which she previously attributed to stress. However, she reports new ulcers in her mouth and new easily-ruptured blisters on her skin. Approximately 6 weeks ago, she reported starting a new drug, captopril, for management of her hypertension. On physical exam, she has dozens of flaccid bullae and erosions on her trunk and extremities; she has 3 ulcers along her buccal mucosa. She is started on systemic steroids and captopril is discontinued.
  • Introduction
    • Clinical definition
      • pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease characterized by painful mucocutaneous lesions
    • Epidemiology
      • demographics
        • most common in
          • those of Mediterranean or Jewish descent
          • 40-60 years of age
    • Pathogenesis
      • autoantibodies (IgG) against desmoglein, a component of desmosome
        • recall that desmosomes connect keratinocytes in the skin
        • type II hypersensitivity reaction
      • this disrupts keratinocyte adhesion and causes separation of epidermis
    • Associated conditions
      • drug use
        • thiols
          • penicillamine
          • benzylpenicillin
          • captopril
          • cephalosporin
      • thymoma
      • myasthenia gravis
      • systemic lupus erythematosus
    • Prognosis
      • without treatment, prognosis is poor
        • 75% mortality at 1 year
      • with treatment, prognosis is improved
        • 10% mortality at 1 year
  • Presentation
    • Symptoms
      • nonhealing and nonscarring ulcers persisting for at least 1 month
        • extremely painful
      • progressive development of blisters from mouth to the rest of the body
    • Physical exam
      • flaccid bullae that are easily ruptured
        • clear exudate
      • superficial erosions of skin (ruptured bullae)
        • often on scalp, face, and trunk
      • mucosal ulcers
      • positive Nikolsky sign
        • extension of blister or sloughing of skin with blunt pressure or lateral traction of skin
  • Studies
    • Labs
      • auto-antibodies detected in serum with enzyme-linked immunosorbent assay (ELISA)
        • anti-desmoglein 3 (mucosal involvement)
        • anti-desmoglein 1 (mucocutaneous involvement)
    • Histology
      • acantholysis
        • separation of epidermal cells
      • intraepidermal blister
      • direct immunofluorescence
        • intercellular deposition of IgG or C3 in a net-like (reticular) pattern
  • Differential
    • Bullous pemphigoid
      • less severe than PV
      • does not affect mucous membranes
      • negative Nikolsky sign
  • Treatment
    • Conservative
      • wound care
        • indications
          • for all patients, to prevent secondary bacterial infection
        • antiseptics
        • anesthetic nebulizers for mucosal lesions
        • potent topical steroids
    • Medical
      • systemic steroids
        • indications
          • mainstay treatment
        • drugs
          • prednisolone
      • steroid-sparing immunosuppressants
        • indications
          • may be used concurrently with steroids for flares refractory to steroids
        • drugs
          • azathioprine
          • mycophenolate mofetil
  • Complications
    • Skin and soft tissue bacterial infection of open lesions
    • Risk of PV in newborn
      • due to placental transfer of maternal autoantibodies
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