Bullous Pemphigoid and Pemphigus Vulgaris

Question 1

Blisters in Bullous pemphigoid arise from the epidermis. TRUE/FALSE?

Answer 1

FALSE
Bullous pemphigoid blisters are SUB-epidermal
=> harder to break

Question 2

Bullous Pemphigoid is an autoimmune condition. What do the antibodies target?

Answer 2

hemidesmosomal proteins BP180 and BP230.

Question 3

Bullous pemphigoid is more common in elderly patients. TRUE/FALSE?

Answer 3

TRUE

Question 4

Common features of bullous pemphigoid

Answer 4

• itchy, tense blisters typically around flexures
• blisters usually heal without scarring
• stereotypically no mucosal involvement (i.e. mouth is spared)

Question 5

What finding on skin biopsy would indicate a diagnosis of Bullous Pemphigoid?

Answer 5

• immunofluorescence shows IgG and C3 at the dermo-epidermal junction

Question 6

Management of Bullous Pemphigoid

Answer 6

oral corticosteroids
ALSO:
- topical corticosteroids
- immunosuppressants
- antibiotics

Question 7

Pemphigus Vulgaris is an autoimmune condition. What are the antibodies directed against?

Answer 7

• Desmoglein 3
  a cadherin-type epithelial cell adhesion molecule.

Question 8

Pemphigus Vulgaris is more common in what population?

Answer 8

Ashkenazi Jewish

Question 9

Features of Pemphigus Vulgaris

Answer 9

• mucosal ulceration (often presenting symptom)
• Oral involvement in 50-70% of patients
• skin blistering - flaccid, easily ruptured
• Lesions are typically painful but not itchy

Question 10

What is Nikolskys sign in Pemphigus Vulgaris?

Answer 10

the spread of bullae
after application of horizontal, tangential pressure to the skin

Question 11

What is typically found on a skin biopsy of a patient with Pemphigus vulgaris?

Answer 11

Acantholysis
- loss of attachments between keratinocytes

Question 12

Management of pemphigus vulgaris

Answer 12

1st Line = steroids
immunosuppressants