Back to Basics: Pemphigus Vulgaris

Pathology: Chronic autoimmune mucocutaneous disease against desmosomes in epidermis


  • painful; rarely pruritic; afebrile
  • flaccid bullae but may start tense, +Nikolsky's sign
  • mucosal involvement common 
  • Bullous pemphigus: Have tense bullae (may start with urticarial lesions), negative Nikolsky's sign, mucosal involvement less likely

Diagnosis: clinical; biopsy is gold standard. 


  • Supportive care
  • Systemic corticosteroids in consult with Rheumatology and/or Dermatology
  • Plasmapharesis or IVIG in severe cases

Prognosis: ~10% mortality secondary to complications of secondary infection and long-term steroid use