Vesiculobullous Disease

Question 1

What are the two types of immunogenic mediated disease?

Answer 1

Cell mediated immunity
Antibody immediate immunity

Question 2

What are some local oral diseases?

Answer 2

Aphthous ulcers
Lichen planus
OFG

All via cell mediated immunity

Question 3

What are some systemic diseases that have local effects on the oral mucosa?

Answer 3

Pemphigoid
Pemphigus
Lupus
Sjogrens
Erythema Multiforme (type 3 hypersensitivity Rxn)

all via antibody mediated immunity

Question 4

What is an antigen?

Answer 4

This is a substance/toxin that triggers the immune system and antibody formation as the body wants to eradicate it

Question 5

What is a self antigen?

Answer 5

This is a normal protein or protein complex produced by the body that the immune system recognises as being harmful and creates an antibody to eradicate it and is the basis of how AI disease works

Question 6

What is an antibody?

Answer 6

This is a protein made my B lymphocytes that bind to antigens to alter or eradicate them

Question 7

What does an autoantibody attack on skin components do?

Answer 7

Causes loss cell adhesion and results in a split forming to which inflammatory exudate can pass and force apart different layers of skin resulting in blister or vesicle formation

Question 8

What do hemidesmosomes to?

Answer 8

Attach epithelial cells to the basement membrane and each other

Question 9

What do desmosomes do?

Answer 9

They attach epithelial cells to each other

Question 10

What is the target antigen in many vesiculobullous diseases?

Answer 10

Desmoglein

Question 11

What is erythema multiforme?

Answer 11

This is an immune mediated condition that can results in target skin lesions, lip crusting, genital lesions, oral ulcerations

more common in younger males

Question 12

How does erythema multiforme occur?

Answer 12

This occurs when antigen antibody react to form a large complex in the circulation that eventually wedges in capillary and results in complement system being activated (triggers inflammation, phagocytes, enhances antibody activity) and as a result perivascular inflammation which results in blisters and ulceration

Question 13

How long does EM last?

Answer 13

usually 2-3 weeks with 1-6 months of disease free period
it has acute onset

Question 14

Where in the mouth can EM affect?

Answer 14

Anywhere including keratinised mucosa

Question 15

What is the cause of the oral lesions in EM?

Answer 15

Often caused by herpes simplex virus reactivation which triggers an immune response leading to a type 3 hypersensitivity rxn

Question 16

What can we do if pt has repeated oral mucosa lesions?

Answer 16

Can give aciclovir prophylactically - 400mg 2x day to suppress herpes simplex replication

Question 17

How do we tx EM?

Answer 17

Hydration
Analgesia
High dose steroid (prednisolone up to 60mg) to suppress the immune system, and complement activation
prophylactic aciclovir if oral lesions

Question 18

What is angina bulls haemorrhagica?

Answer 18

This is tight blood filled blisters in the oral mucosa that are benign and often causes by trauma during eating

Pt may present with painless blood filled blister or may have burst and present as ulceration that often is only mildly symptomatic for one day

Question 19

Where is angina bullosa haemorrhagica often seen?

Answer 19

Buccal mucosa
Soft palate

Question 20

What is the onset of ABH?

Answer 20

acute, rapid consent, often appears in few mins last one hour before bursting to form ulcer

Question 21

Do we do any tests for ABH?

Answer 21

NO - ulcer comes back as non specific ulceration and negative test on immunofluorescence

Question 22

What is the tx for ABH?

Answer 22

CHX - 0.2% 10ml 1min 2 x day

Diflamm - 0.15% mouth rinse that has anti-inflame and local analgesics

Question 23

What is pemphigoid?

Answer 23

This is a condition where antibodies target hemidesmosomes and results in itchy raised rash which progresses to large blisters (more common in elderly pts)

Question 24

How does pemphigoid occur?

Answer 24

This is where the antibody attacks hemidesmosomes resulting in separation of the epithelium at the basement membrane and fluid and inflammatory exudate fills the space between the epithelium and BM resulting in large blisters forming

sub-epithelial so more resistant to bursting and often pt will present with blisters intact

Question 25

How will pt with pemphigoid present?

Answer 25

Blistering problem that lasts in ruptured areas there is exposed CT and potential infection resin and fluid exudate dehydration and infection risk may be scarring if cicatritial pemphigoid - can cause narrowed oropharynx, scarred conjunctivae

Question 26

Tyoes of pemphigoid?

Answer 26

Bullous pemphugoid = skin Mucous membrane pemphigoid = eyes, genitals, mouth Cicatritial pemphigoid = scarring type

Question 27

How do we test for pemphigoid?

Answer 27

Gold standard is DIRECT IMUNOFLUORESCENCE - we take a peri-lesional sample (as if we take sample of lesion only we may not get good enough sample for testing) Result will show LINEAR BASEMENT STAINING (as there is separation between the epithelium and CT but in perilesions sample we see staining along BM) also presence of IgG and C3

Question 28

How do we manage pemphigoid?

Answer 28

Steroid tx for immunosuppressant activity (prednisolone) Immune modulation drugs such as aziothoprine

Question 29

What is pemphigus?

Answer 29

This is an immune mediated antibody disease where antibody attacks the desmosomes resulting in painful life threatening blisters which burst leaving erosive area and mucosla surface loss

Question 30

How does pemphigus occur?

Answer 30

It occurs due to antibody attacking desmosomes which attach epithelial cells together - the antibodies attack all around the cell results in epithelial cells losing adhesion and fluid exudate intra epithelially (less sub epithelial than pemphigoid so often blisters tend to burst) Often only a few layers of cells around lesion as as the fluid exudate fills the space there is loss of epithelium - erosion of loss of mucosal coverage

Question 31

If pt presents with intact blisters what do they not have?

Answer 31

thet dint have pemphigus - as blisters are fragile and burst easily and would present with erosion and loss of mucosal surface

Question 32

Where is often affected in pemphigus?

Answer 32

Skin - 3 yrs after mucosa - initially

Question 33

How is pemphigus treated?

Answer 33

if no tx = fatal high dose steroids immune modulating drugs = aziothoprine