Pemphigus Vulgaris

Question 1

What is the pathophyiology of Pemphigus Vulgaris?

Answer 1

It is an autoimmune disease caused by antibodies directed against desmoglein 3, a cadherin-type epithelial cell adhesion molecule.

Question 2

What is usually the most common presenting feature and in what % of people is it seen in?

Answer 2

Mucosal ulceration is common and often the presenting symptom.

Oral involvement is seen in 50-70% of patients

Question 3

What are some other features of pemphigus vulgaris?

Answer 3

• Skin blistering - flaccid, easily ruptured vesicles and bullae.
• Lesions are typically painful but not itchy. These may develop months after the initial mucosal symptoms. Nikolsky’s describes the spread of bullae following application of horizontal, tangential pressure to the skin
• Acantholysis on biopsy

Question 4

How is pemphigus vulgaris treated?

Answer 4

• Immunosupressants
• Steroids

Question 5

What is the difference between bullous pemphigoid and pemphigus vulgaris?

Answer 5

• Pemphigus Vulgaris
  
  • Oral involvement
  • Younger patients
  • epidermal layer. (U=Upper)
  • Blisters rupture easily
  • Most patients die without treatment.
• Bullous pemphigoid
  
  • No oral involvement
  • Older patients
  • supepidermal blisters (D=dermal)
  • Blisters are tense and firm
  • Prognosis is good

Question 6

Answer 6

Question 7

Question 8

What population is pemphigus vulgaris more commonly seen in?

Answer 8

Young Ashkenazi Jews