Pemphigus and Pemphigoid

Question 1

What is Bullous Pemphigoid?

Answer 1

Autoimmune aetiology causing sub-epidermal blistering of the skin
Antibodies against proteins BP180 and BP230 produced

Question 2

What are clinical features of Bullous Pemphigoid?

Answer 2

Onset around age 6-80
Large tense bullae- some erupt and form crusted erosions, others heal without scarring
Itchy, tense more commonly found around flexures
NO mucosal involvement

Question 3

What investigations are required for Bullous Pemphigoid?

Answer 3

Biopsy- IgG antibodies and C3 at dermo epidermal junction

Question 4

What is the management of Bullous Pemphigoid?

Answer 4

Refer to dermatology
Oral steroids= mainstay
Can use topical steroids, immunosuppressants

Question 5

What is Pemphigus Vulgaris?

Answer 5

Autoimmune disease caused by antibodies against desmogelin 3
More common in Ashkenazi Jewish population and Indian population

Question 6

What are clinical features of Pemphigus Vulgaris?

Answer 6

Affects people aged 40-60
Commonly starts with mucosal ulceration either on mucous membranes, inside the mouth or around genitals
Tense blisters
- rupture easily
- painful but not itchy

Question 7

What are investigations of Pemphigus Vulgaris?

Answer 7

Biopsy
- acantholysis- loss of coherence between epidermal cells due to destruction of desmoglein molecules

Question 8

What is the management of Pemphigus vulgaris?

Answer 8

Dermatology referral
Oral steroids= 1st line
Immunosuppressants can be considered