Pemphigus

Question 1

What is pemphigus?

Answer 1

An autoimmune condition resulting in supra basilar split in epithelium

Question 2

What is the pathophysiology of pemphigus?

Answer 2

Auto antibodies against desmoglein 1 and 3, activates complement, causing acantholysis and blister formation

Question 3

Where are Dsg 1 proteins found

Answer 3

In skin

Question 4

Where are Dsg 3 proteins found?

Answer 4

In mucous membrane (oral cavity)

Question 5

What is the function of desmosomes?

Answer 5

Intercellular adhesion

Question 6

What is the presentation of pemphigus?

Answer 6

Thin walled blisters which easily rupture (pt often wont notice the blister as happens so quickly)
Ragged ulceration
Mucosal atrophy
Rarely affects attached gingivae

Question 7

What investigations should be carried out if pemphigus is suspected?

Answer 7

Take 2 biopsy for H&E staining and direct immunofluoresence

Question 8

What are the findings of H&E staining in pemphigus?

Answer 8

Supra basilar split in epithelium
Acantholysis
Tzank cells

Question 9

What are the findings of DIF in pemphigus?

Answer 9

IgG or C3 proteins deposited intercellularly - basket weave appearance

Question 10

What is the gold standard test for pemphigus diagnosis and why?

Answer 10

DIF - shows immunoglobulin subclass and binding pattern

Question 11

What is the treatment for pemphigus?

Answer 11

Symptomatic relief - benzydamine mouthwash
Topical steroids - betamethasone mouthwash, orobase gel
Systemic immune modulators - eg aziathioprine (allows for reducing dose of steroids)
Systemic steroids - prednisone (initially 10-20mg, increase gradually).