Updated: 2/1/2022

Pemphigus Vulgaris

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  • Snapshot
    • A 50-year-old man presents to his dermatologist for persistent blisters. He reports that the blisters started on his lips 3 weeks ago. He continues to develop new painful lesions on his lips but also on his trunk and arms. His dermatologist performs a punch biopsy. In the meantime, he is given topical steroids and an anesthetic for his mucosal lesions.
  • Introduction
    • Clinical definition
      • pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease characterized by painful mucocutaneous lesions
    • Associated conditions
      • drug use
        • thiols
          • penicillamine
          • benzylpenicillin
          • captopril
          • cephalosporin
      • thymoma
      • myasthenia gravis
      • systemic lupus erythematosus
  • Epidemiology
    • Demographics
      • most common in
        • those of Mediterranean or Jewish descent
        • 40-60 years of age
  • ETIOLOGY
    • Pathogenesis
      • autoantibodies (IgG) against desmoglein, a component of the desmosome
        • recall that desmosomes connect keratinocytes in the skin
        • type II hypersensitivity reaction
      • this disrupts keratinocyte adhesion and separation of the epidermis
  • Presentation
    • Symptoms
      • nonhealing and nonscarring ulcers persisting for at least 1 month
        • extremely painful
      • progressive development of blisters from mouth to elsewhere
    • 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
        • anti-desmoglein 1
    • Histology
      • acantholysis
        • separation of epidermal cells
      • intraepidermal blister
      • direct immunofluorescence
        • intercellular deposition of IgG or C3 in 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
  • Prognosis
    • Without treatment, prognosis is poor
      • 75% mortality at 1 year
    • With treatment, prognosis is improved
      • 10% mortality at 1 year
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