Vesiculobullous disease

Question 1

Name 3 immunological oral diseases that are local to the oral cavity

Answer 1

Aphthous ulcers
Lichen planus
Orofacial granulomatosis

Question 2

Name 6 systemic diseases that have local effects on the oral mucosa (immunological oral diseases)

Answer 2

Erythema Multiforme
Pemphigus
Pemphigoid
Lupus erythematosis
Systemic Sclerosis
Sjogren’s Syndrome

Question 3

What type of immunity are aphthous ulcers, lichen planus and OFG?

Answer 3

Cell-mediated immunity

Question 4

How are blisters formed in immunological skin diseases?

Answer 4

Auto-antibody attack on skin components causing loss of cell-cell adhesion
Split forms in skin - fills with inflammatory exudate and forces layers of skin apart = blister is formed.

Question 5

What technique is used for diagnosing vesiculobullous condition?

Answer 5

Direct immunofluorescence

Question 6

What technique is used for monitoring vesiculobullous diseases?

Answer 6

Indirect immunofluorescence
Not always useful for diagnosis

Question 7

What is erythema multiforme?

Answer 7

A spectrum auto-immune disease considered to be a reaction to an outside stimulus such as infection or medication.

Question 8

What type of hypersensitivity reaction is erythema multiforme?

Answer 8

Type 3 - reaction mediated by immune complexes

Question 9

What is the urgent medical treatment for oral lesions of erythema multiforme?

Answer 9

Systemic steroids - up to 60mg/day (high dose)
Systemic aciclovir - secondary to need for steroids

Question 10

What should you prescribe for recurrent problems of erythema multiforme?

Answer 10

Prophylactic aciclovir - 400mg twice daily

Question 11

What is general management for erythema multiforme?

Answer 11

Encourage fluid intake
Encourage analgesia
Allergy test - wide variety of environmental triggers

Question 12

What is angina bullosa haemorrhagica?

Answer 12

Blood-filled tight blisters on the oral mucosa - most common oral blistering condition

Buccal mucosa and soft palate are most common sites
Rapid onset
Last about 1 hour then burst

Question 13

What is management for angina bullosa haemorrhagica?

Answer 13

Symptomatic relief rather than exact treatment - CHX mouthwash or benzydamine (difflam) spray.

Question 14

To diagnose WHICH condition would you want to take a PERI-LESIONAL BIOPSY?

Answer 14

Pemphigoid

If you take biopsy of blister - often no epithelium will be found.

Question 15

What is the most common vesiculobullous condition in primary care?

Answer 15

Pemphigoid

Question 16

What are the clinical differences between pemphigus vulgaris and mucous membrane pemphigoid?

Answer 16

Pemphigus affects the outer layer of the skin (epidermis) and causes lesions and blisters that easily rupture- mucosal ulceration/ erosion.
Pemphigoid affects a lower layer of the skin, between the epidermis and the dermis, creating tense blisters that do not break easily.

Question 17

What would typically be seen in direct immunofluorescence for someone with pemphigoid?

Answer 17

Linear staining along the basement membrane
C3 and IgG detected in this area in ‘standard’ pemphigoid

Question 18

What would typically be seen in direct immunofluorescence for someone with pemphigus?

Answer 18

Basket-weave pattern surrounding each of the epithelial cells.

Question 19

What are key characteristics of pemphigus?

Answer 19

RARELY see intact bullae - eroded mucosa and loss of epithelial covering. Surface easily lost.

Typically oral lesions occur before skin.

Question 20

What are the commonest antibodies detected in pemphigus and pemphigoid?

Answer 20

C3 and IgG

Question 21

What are tzank cells and where are they characteristically seen in?

Answer 21

Broken off epithelial cells due to weakened attachment - seen in histopathology for pemphigus

Question 22

What does the ELISA test do in pemphigus vulgaris?

Answer 22

Detects antibodies against desmoglein 1 and desmoglein 3

Question 23

What virus is erythema multiforme closely associated with?

Answer 23

Herpes simplex virus

Question 24

What type of hypersensitivity reaction are pemphigus vulgaris and mucous membrane pemphigoid?

Answer 24

Auto-immune type II hypersensitivity reaction

Question 25

What are the histological difference between pemphigus and pemphigoid?

Answer 25

Pemphigus - SUPRA-BASAL split. Autoantibodies attack DESMOSOMES. Presence of Tzank cells

Pemphigoid - SUB-EPITHELIAL split, autoantibodies attach HEMIDESMOSOMES

Question 26

What is the topical management for pemphigus and pemphigoid?

Answer 26

betamethasone mouthwash (0.5mg 2-3x/ day
Beclomethasone inhaler 50mg 2-3x/ day

Question 27

What is the systemic management of pemphigus and pemphigoid?

Answer 27

Systemic steroids
Immunomodulators - azathioprine, mycophenolate

Question 28

What are the different types of pemphigoid?

Answer 28

Bullous - skin
Mucous membrane pemphigoid - ALL - eye, genital, oral
Cicatricial pemphigoid - mucosal with SCARRING

Question 29

What is the difference between DIRECT immunofluorescence and INDIRECT immunofluorescence?

Answer 29

DIRECT - most useful test of vesiculobullous condition. Antibody bound tissue targeted in DIF

INDIRECT - circulating antibody not yet bound to tissue
Not always useful for diagnosis - often good for monitoring disease activity