Vesiculobullous lesions 1

Question 1

What are the immune mediated diseases with local effects on the mouth?

Answer 1

Aphthous ulcers

LP

OFG

Question 2

What are the systemic immune mediated diseases which can also appear in the mouth?

Answer 2

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

Question 3

What type of hypersensitivity reaction is associated with erythema multiforme?

Answer 3

Type 3- related to antigen-antibody complexes

Question 4

What immunogenic oral diseases are cell mediated?

Answer 4

Aphthous ulcers

Lichen Planus

Orofacial Granulomatosis

Question 5

What immunogenic oral diseases are antibody mediated?

Answer 5

Pemphigus

Pemphigoid

Question 6

Why are skin and oral immunological diseases related?

Answer 6

Often affects both due to common embryological origin

Question 7

What is the difference between an antigen and an epitope?

Answer 7

Antigens- big immunogenic sites within protein

Epitope is the small part in sequence of protein which antibody binds to

-> Binding at these can affect conformation of protein

Question 8

How does antibody binding to different epitopes affect disease course in Epidermolysis Bullosa?

Answer 8

Depending on epitope it can be mild or lethal form

Question 9

How does damage occur in immunological skin diseases?

Answer 9

Auto-antibody attack on skin components (hemi-/desmosomes) causing loss of cell-cell adhesion

‘Split’ forms in skin
-> Fills with inflammatory exudate (forces apart layers)
-> Forms vesicle/blister

Question 10

Which protein is targeted within desmosomes in immunobullous diseases?

Answer 10

Desmoglein

Question 11

What is the difference between a vesicle and a blister?

Answer 11

Vesicle- 1-2mm

Blister- larger

Question 12

How does direct immunofluorescence used to identify antibodies causing immunological conditions?

Answer 12

Secondary antibody with fluorescein binds to the primary antibody which is bound in the tissue
-> can be seen under fluorescent light

Question 13

What are the requirements for DIF?

Answer 13

Sample must not be put into formalin containing transport medium as it causes loss of binding sites

Must be transported fresh and processed quickly

Question 14

When is indirect immunofluorescence used?

Answer 14

When identifying a circulating antibody which is not bound in tissue
-> done using plasma samples

Less reliable for diagnosis but good for monitoring disease

Question 15

Which disease is indirect immunofluorescence useful for?

Answer 15

Pemphigus- as antibody levels direct treatment need

Question 16

What is erythema multiforme?

Answer 16

Spectrum disorder of Immunogenic related skin and mucosa ulceration
-> Variable orofacial involvement

Question 17

What causes Erythema multiforme to occur?

Answer 17

 Antigen presents which is targeted by antibody (antigen is usually one that has been met before and prompt immune response follows- antibodies produced by memory B cells)

 Antigen and antibody combine to form complexes in circulation but cannot pass through capillaries, it can become wedged in tissue and results in activation of complement within BV causing perivascular inflammatory response (If significant- blistering and ulceration of tissue presents)

Question 18

How does EM present in skin and mucosa?

Answer 18

Skin- target lesion/crusting lesions

Mucosa- ulcers
-> present similar to primary herpetic gingivostomatitis (can affect keratinised tissue)

Lesions tend to last for 2-3 weeks, there may be months between episodes or they can develop very quickly

Question 19

What are the causes of EM?

Answer 19

Drugs

HSV reactivation triggering T3 rxn

Mycoplasma- infective agent

Question 20

What areas can EM affect?

Answer 20

Mouth

Skin

Eyes- conjuctivae

Genitals

If multi-system and severe- it is known as Steven Johnson Disease

Question 21

How is oral lesions in EM managed?

Answer 21

Drugs
-> systemic steroids – up to 60 mg/day (a high dose)
-> systemic aciclovir

Encourage fluid intake- May require admission for IV fluid if unable to drink due to discomfort

Encourage analgesia

Question 22

How is recurrent EM managed?

Answer 22

Consider prophylactic aciclovir daily

Allergy test – a wide variety of environmental triggers

Question 23

What is the dose of prophylactic aciclovir for EM?

Answer 23

400mg x 2 daily
-> helps supress virus replication and prevent emergence of EM and production of antibodies/immune complexes

Question 24

What is angina bullosa haemorrhagica?

Answer 24

Angina- tight, Bullosa- blister, haemorrhagic- blood filled
-> painless blood blisters which occur in the mouth

Question 25

What are the most common sites for ABH?

Answer 25

Buccal mucosa

Soft palate
-> sometimes occurs at junction of soft and hard palate and can feel like airway is closing

Question 26

What are the causes of ABH?

Answer 26

Minor trauma- can be from eating

Steroid inhalers

Question 27

How long do ABH lesions last?

Answer 27

Rapid onset- form within minutes

Burst within one hour
-> may leave mildly symptomatic ulcer

Question 28

What do healing ABH lesions look like?

Answer 28

Aphthous ulcers- to differentiate ask if the lesion was previously a blister

Question 29

What should patients be informed if they are worried about the colour of the liquid that comes out on bursting of ABH blister?

Answer 29

That it is fluid that is blood stained due to RBC presence within rather than any worrisome bleeding
-> not related to platelet/coagulation defect

Question 30

What does ABH come up as on histological testing?

Answer 30

Non-specific ulceration

• also negative to DIF/IF testing

Question 31

What can be used to treat symptoms of ABH?

Answer 31

CHX mouthwash

Difflam mouthwash