Pathology: Chronic autoimmune mucocutaneous disease against desmosomes in epidermis

Clinical:

• 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. 

Management:

• 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