Pemphigus vulgaris

Question 1

What is pemphigus vulgaris?

Answer 1

One of the autoimmune blistering conditions characterised by flaccid intra-epidermal blisters.

Question 2

Aetiology of pemphigus vulgaris?

Answer 2

Autoimmune

Usually presents in middle age (40-60)

Question 3

Pathophysiology of pemphigus vulgaris?

Answer 3

Type 2 hypersensitivity - IgG4 antibodies against desmosomal proteins leads to a loss of keratinocyte adhesion.

Question 4

Clinical symptoms of pemphigus vulgaris?

Answer 4

Produces fluid-filled, painful, fragile blisters.

Blisters rupture to form shallow erosions

Common sites include scalp, face, axillae, groin and trunk.

May also affect the mucosa e.g. mouth and respiratory tract.

Question 5

Clinical signs of pemphigus vulgaris?

Answer 5

Blisters will be nikolsky sign positive - blisters can be extended with gentle lateral pressure.

Question 6

Investigations for pemphigus vulgaris?

Answer 6

Biopsy:
- Immunofluorescence: chicken wire deposition of IgG within th epidermis.

• Histology: intra-epidermal blister with accumulation of inflammatory cells within the dermis.

Question 7

Management of local disease?

Answer 7

Topical steroids + topical anaesthetics

Question 8

Management of systemic disease?

Answer 8

High dose oral steroids (prednisolone) + immunosuppression +/- rituximab

• Most patients require oral steroids as patients are often very unwell.