Pemphigus (Vulgaris)

Question 1

What is pemphigus?

Answer 1

Pemphigus is a rare, autoimmune bullous disease, in which there is a loss of integrity of epidermal cell adhesion

Question 2

What are the 4 distinct subtypes of pemphigus?

Answer 2

• Pemphigus vulgaris (80%)
• Pemphigus foliaceus
• IgA pemphigus
• Paraneoplastic pemphigus

Question 3

What is pemphigus vulgaris?

Answer 3

This is an autoimmune immunobullous condition in which desmosomes are targeted, leading to breakdown of the epidermis

Question 4

Which age group is most commonly affected by pemphigus vulgaris?

Answer 4

Middle age

Question 5

Describe the pathophysiology of pemphigus vulgaris

Answer 5

This is an autoimmune condition in which IgG auto-antibodies are made against desmoglein-3

Desmoglein-3 maintains desmosomal attachments between the cells of the epidermis

Immune complexes begin to form on the cell surface, activating the complement system and releasing proteases

This disrupts desmosomes, resulting in acantholysis (Breakdown of the epidermis)

Question 6

Where does pemphigus vulgaris most commonly occur?

Answer 6

This will present on the skin of the scalp, face, axilla, groin and trunk and may affect the mucosa such as the mouth, eyes, oesophagus, vulva, rectum and respiratory tract

Question 7

How does pemphigus vulgaris usually present?

Answer 7

Formation of flaccid vesicles or bullae, which can rupture easily, leading to erosions

It often affects the mucosa first before the skin, with oral mucosal involvement in up to 70% of cases

Nikolsky sign will be positive (Lateral pressure on the skin next to the blister causes it to extend)

Question 8

What condition is shown?

Answer 8

Pemphigus vulgaris

Question 9

From where is a biopsy of a lesion taken in pemphigus vulgaris?

Answer 9

From across the edge of the lesion, getting both normal and abnormal skin

Question 10

What test is performed on the normal skin of a pemphigus vulgaris biopsy?

Answer 10

Immunofluorescence

Question 11

What test is performed on the abnormal skin of a pemphigus vulgaris biopsy?

Answer 11

H and E staining

Question 12

What will H and E staining of pemphigus vulgaris show?

Answer 12

Question 13

What will immunofluorescence in pemphigus vulgaris lesions show?

Answer 13

IgG in Chicken wire pattern

Question 14

How is pemphigus vulgaris treated locally?

Answer 14

Topical steroids
Anaesthetics

Question 15

How is pemphigus vulgaris treated systemically?

Answer 15

• Prednisolone ±
  
  • Azathioprine
  • Dapsone
  • Ciclosporin
  • Plasmapheresis
  • Rituximab

Question 16

How long will most cases take to remit?

Answer 16

3-6 years