Updated: 11/29/2018

Pemphigus Vulgaris

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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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