Pemphigus vulgaris

Background

• Chronic autoimmune mucocutaneous disease against desmosomes in epidermis (bind keratinocytes)

Clinical Features

• Painful but rarely pruritic
• Mucosal involvement common
  • Presenting complaint in 50% of cases.
• Primary lesions
  • Tense and clear vesicles/bullae on head, trunk, mucosa.
  • Become flaccid and turbid 2-3 days later
  • Rupture and leave sensistive denuded area of skin
    • Slow to heal and prone to secondary infection.
• Nikolsky's sign: Sliding pressure applied to normal skin adjacent to blister causes further ulceration.
  • Intraepidermal acantholyis: Keratinocytes separated at the basal layer from one another.
  • Gives appearance of lesion expanding into adjacent tissue.
  • Transudate accumulate between keratinocyte and basement membrane which gives rise to new blisters.

Differential Diagnosis

Bullous Rashes

• Bullous pemphigoid
• Cicatricial pemphigoid
• Dermatitis herpetiformis
• Drug-Induced Bullous Disorders
• Erythema multiforme
• Pemphigus Vulgaris

Diagnosis

• Clinical diagnosis. Nikolsky's sign may be helpful to differentiate from other bullous diseases.
  • Gold standard: punch biopsy

Management

• IVF and electrolyte resuscitation
• Systemic Corticosteroids
• IV Antibiotics for signs of secondary infection.
• Consider need for Rheumatology evaluation
  • Plasmapharesis and IVIG in severe cases.

Disposition

• Admit if evidence of:
  • Significant fluid or electrolyte loss
  • Decompensated disease
  • Burn unit for large BSA involvement