Pemphigus Vulgaris

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

Treatment

Complications