Oral blistering diseases

Question 1

What are the two types of immunogenic reactions?

Answer 1

• Cell mediated
• Antibody mediated

Question 2

What type of hypersensitivity reaction happens in Erythema multiforme?

Answer 2

Type 3

Question 3

What 3 diseases are related to cell mediated immunity?

Answer 3

• Apthous ulcers
• Lichen planus
• Orofacial granulomatosis

Question 4

What 2 diseases is related to antibody mediated immunity?

Answer 4

• Pemphigus
• Pemphigoid

Question 5

Why might oral disease and genital/skin are commonly linked?

Answer 5

Because they share many common antigens and epitopes

Question 6

What are antigens?

Answer 6

• Immunogenic site within a protein

Question 7

What is epitope?

Answer 7

• A specific site in an antigen that antibodies bind

Question 8

Describe the immunological process in blistering disease?

Answer 8

Auto-antibody attack on skin component cause loss of cell to cell adhesion resulting in a split which is filled by inflammatory exudate forming a vesicle or blister

Question 9

What is the difference between a vesicle and a blister?

Answer 9

• Vesicle 1-2mm
• Blisters are larger

Question 10

What exactly do autoantibodies attack that causes loss of cell to cell adhesion?

Answer 10

Desmosomes and Hemidesmosomes

Question 11

Briefly describe what is a desmosome and a hemidesmosome?

Answer 11

Desmosome - an attachment that bind cells together
Hemidesmosome - anchor cells to basement membrane

Question 12

Describe how direct immunofluorescence work?

Answer 12

• by manufacturing an antibody with a flour-scene marker that binds to the primary antibody in the affected tissue , confirming its presence

Question 13

How should the tissue for direct immunofloursence be transported?

Answer 13

Fresh , avoid formalin as it may cause the loss of binding sites

Question 14

What is indirect fluorescence? and when to use it?

Answer 14

• it confirms the presence of antibodies circulating in the plasma that is not yet bound to the tissue
• not reliable as a diagnostic aid but can be used for monitoring

Question 15

What is erythema multiforme?

Answer 15

A spectrum disorder of immunogenic related skin and mucosa ulceration and blistering

Question 16

What do we call the severe form of erythema multiforme?

Answer 16

Stevens-johnson-syndrome

Question 17

3 parts of the body other than the mouth that can be affected by stevens-johnson syndrome?

Answer 17

• Skin
• Eyes
• Pharynx /nose

Question 18

3 contributing things to the development of erythema multiforme?

Answer 18

• drugs
• herpes simplex
• mycoplasma

Question 19

Which 2 parts of the mouth do erythema multiforme commonly affect?

Answer 19

• Lip and anterior parts of the mouth

Question 20

What 3 signs might be associated with erythema multiforme?

Answer 20

• Crops of ulcers in the mouth that heal within 2 weeks
• Dysphagia
• Dehydration

Question 21

What is the emergency management of erythema multiforme?

Answer 21

• high dose systemic steroids (prednisolone)
• systemic aciclovir

Question 22

What advice would you give to a patient with erthema multiforme?

Answer 22

• increase fluid intake
• analgesia

Question 23

Incidence or erythema multiforme?

Answer 23

Young males

Question 24

What is advised when if there is recurrent problems with erythema multiforme ?

Answer 24

• prophylactic acivlovir daily
• allergy testing

Question 25

What is the most common oral blistering condition?

Answer 25

* Angina bullosa haemorrhagica which is a blistering disease affecting the mouth that presents as blood filled blisters that burst within an hour leaving a relatively painless vesicle * Sudden onset * intermittent

Question 26

What are angina bullosa haemorrhagica commonly called?

Answer 26

blood blisters

Question 27

What are the commonest sites for angina bullosa haemorrhagica ?

Answer 27

Buccal mucosa and soft palate

Question 28

What are 2 triggers of ABH?

Answer 28

* Trauma * Eating

Question 29

When do ABH heal?

Answer 29

Heal within days without scarring

Question 30

What advice would you give a patient with ABH?

Answer 30

Use chlorhexidine mouthwash and reassure patient that it is benign

Question 31

What is pemphigoid?

Answer 31

An autoimmune blistering disease characterised by thick walled blisters which can be filled with blood or clear fluid affecting the full epidermis

Question 32

What are the three forms of pemphigoid?

Answer 32

* Bullous pemphigoid - skin * Mucous membrane pemphigoid - all mucous membranes * Circatritial pemphigoid - mucosal with scarring

Question 33

What is the histopathological feature important in diagnosing pemphigoid?

Answer 33

Sub epithelial split at the epithelial/connective tissue junction involving hemidesmosomes at basement membrane

Question 34

Describe the immunofluorescence appearance in pemphigoid?

Answer 34

* Linear staining along the basement membrane * C3 and IgG detected in this area

Question 35

Other than C3 and IgG what other antibodies can be found in pemphigoid?

Answer 35

IgA - linear staining (linear IgA disease)

Question 36

In what disease is there granular IgA and C3 staining?

Answer 36

Dermatitis herpetiformis

Question 37

What symptom/sign of pemphigoid is seen in the eyes?

Answer 37

Symblepharon

Question 38

How can pemphigoid be managed?

Answer 38

* steroids - PREDNISOLONE * immune modulating drugs

Question 39

What is the commonest form pemphigus?

Answer 39

Pemphigus vulgaris which is an autoimmune blistering disease characterised by clear blisters that burst and spread which can be related to genetic factors

Question 40

What are two common histological signs of pemphigus?

Answer 40

* Suprabasal split * Tzank cells

Question 41

What pattern of immunofluorescence is seen in pemphigus?

Answer 41

Basket weave pattern C3 and IgG

Question 42

Which to proteins are associated with pemphigus vulgaris?

Answer 42

Desmoglein 1 and 3

Question 43

What two proteins are associated with pemphigoid ?

Answer 43

BP180 and BP230