Vesiculobullous Diseases

Question 1

Why is it often diseases are seen to affect both the skin and the oral mucosa

Answer 1

This is because embryologically the oral mucosa develops from the same precursor as the skin, they share many common antibodies and epitopes, so when antigens and diseases affect one it tends to affect both

Question 2

Why do vesicle/ blisters form in immunological skin disease?

Answer 2

Auto-antibody attack on skin components causing loss of cell- cell adhesion
Split forms in the skin which fills with inflammatory exudate forming a small blister

Question 3

What is the difference between a vesicle and a blister

Answer 3

The size of the lesion
Vesicles are typically 1-2mm larger and blisters are larger

Question 4

Discuss desmosome/ hemidesmosome attachment in relation to immunobullous disease and how blisters/vesicles happen

Answer 4

The desmisomes or hemidesmosomes attach the epithelial cells to each other, to the basement membrane and to the protein desmoglein

This protein is the target site for many antibodies involved in immunobullous diseases and the way this is bound to the antibody causes there to be adhesion lost between the desmosomes allowing the cell to split

Question 5

How can the process of the desomosome split be seen?

Answer 5

Direct immunofluresence

Applying tests to biopsy site which are looking for evidence of the antibody within the skin tissues.

Question 6

How does direct immunofluroescnce work

Answer 6

Antibody has been in circulation binding to the tissue causing disease.
By manufacturing another antibody which has got a fluroscceine marker on it, this second antibody will bind to the primary antibody so that when the tissue is examined with lighting the fluorescence will be demonstrated showing where antibody is found in the tissue

Question 7

If a sample taken from a biopsy from immunoflorsence is being transported what must you be wary of?

Answer 7

It must not be put in a FORMALIN containing transport medium as this will cause the binding site to be lost

Must be transported fresh to lab and processed quickly

Question 8

What is indirect immunofluoresnce

Answer 8

In this case the circulating antibody is not yet bound to the tissue

Detected by immunofluorescene from a PLASMA sample

Question 9

Indirect immunoflurescence is not always useful for diagnosis but what can it be useful. For

Answer 9

Often good for monitoring disease activity

Question 10

What is erythema multiformE?

Answer 10

Spectrum disorder of immunogenic related skin and mucosa ulceration

Question 11

How does erythema multiforme happen?

Answer 11

Immune mediated hypersensitivity reaction

Antigens and antibodies combine within the circulation and this large complex is unable to pass within the capillaries it becomes wedged and activates compliment within the tissues causing a peri vascular inflammatory response

When this is signifant the patient will get blistering or inflammation of the tissue

Question 12

What is erythema multiforme called in its most extreme form

Answer 12

Steven Johnsons syndrome

Severe multi system involvement

Question 13

What is erythema Multiforme called in its most extreme form

Answer 13

Steven Johnson’s syndrome

Severe multusstem involvemeeent

Question 14

What are the major attacks in erythema multiforme though to be triggered by

Answer 14

Recurrent hermetic lesions or other infections e.g. mycoplasma

Question 15

What is the most efffective Treatment from erythema multiforme

Answer 15

High dose prednisolone to suppress the antibody antigen reaction and the compliment activation in the tissues

Question 16

Why is hydration so important in patients with erythema multiforme

Answer 16

If patients present late the key thing is hydration as most of these patients present in hospital admission because mouth and pharynx are too sore for fluid intake

Question 17

What is some typical presentation of erythema multiforme

Answer 17

Crusted haemorrhagic Bullae are often seen on the lips, the oral mucosa, eyes and genital skin area.

Target lesions sometimes occur on the skin

In severe illness there may be febrile illness or hospital. Admission

Question 18

What can be prescribed propholactivly for erythema multiforme if recurrent probelms

Answer 18

Acicolvir 400mg 2x daily

To prevent recurrence even in the absence of preceding herpetic infection

Question 19

What is the most common oral blistering condition

Answer 19

Angina bullosa haemorrhagica

‘’Blood blisters ‘’n the mouth

Question 20

What are the most commonly affected sites in angina bullosa haemorrhagica

Answer 20

The hard and soft palate

They suddenly appear( rapid onset) and can reach up to 2am

Question 21

What is the aetiology of angina bullosa haemorrhagica

Answer 21

Relatively painless
No obvious trigger
Heal with no scarring within a few days

Question 22

What must be ruled out before you can reassure a patient that the condition - angina bullosa haemorrhagica is harmless

Answer 22

Must rule out any autoimmune or bleeding Dorset’s

Question 23

What risk factors for angina bullosa haemorrhagica would you report to a pt when giving advice surrounding the condition

Answer 23

Associations with the use of a steroid in her with a nebuliser
The eating of rough foodstuffs
Taking of very hot drinks

Question 24

How can we manage angina bullosa haemorrhagica

Answer 24

There is no treatment available a present
Reassurance to the patient that it is begin
Explain known triggers and course of the disease
They can be annoying but do not progress systemically

If there is discomfort an analgesic mouthwash such as benzydamine hydrochloride may be effective

Question 25

What kind of antibody attack happens in pempigoid?

Answer 25

A sub epithelial antibody attack

The antibodies cause separation from the basement membrane at the connective tissue and inflammatory exudate fills the space

Question 26

What is the difference between mucous membrane pemphhigoid and cicatritial pemphigoid

Answer 26

In Cicatritial pemphiogid there is scarring

Question 27

Why is there infection risk in pemphigoid

Answer 27

In areas of blister rupture there is exposed connective tissue and fluid from the area, this loss of epithelial barrier leads to infection risk

Question 28

In pemphigoid, other than infection risk, what can leaking blisters lead to?

Answer 28

Dehydration

Question 29

What is symblepharon

Answer 29

This happens in pemphigoid
It is when there is scarring in the eye, joining the conjunctiva too the eye surface \

Question 30

Why when assessing pemphigoid or pemphigus must a perilesional biopsy be taken rather than the lister itself

Answer 30

This is because due to the damage to the epithelium in these conditions not enough epithelium will be present in the sample to make a diagnosis

Question 31

When looking an immunofluorsence image, you see linear staining along the basement membrane what would this suggest?

Answer 31

Pemphigoid

Question 32

What will be detected in immunofluoresnce along the basement membrane in standard pemphigoid

Answer 32

C3 and IgG

Question 33

Why do we use immunosuppressant drugs such as azathioprine to manage pemphigoid and pemphigus

Answer 33

To try and prevent the antibody generation which is causing the disease

Question 34

What is the commonest form of pemphigus

Answer 34

Pemphigus vulgaris

Question 35

What happens in pemphigus

Answer 35

An intraepitheial bullae forms

The desmosomes are attacked by the antibodies causing loss of adhesion
As the fluid fills in adhesion is completely lost cashing thinning and eventual loss of the epithelium

Blisters- burst- spread

Question 36

Are you more likely to see intact bullae in pemphigoid or pemphigus

Answer 36

Pemphigoid

Question 37

What condition are Tzank cells characteristic of

Answer 37

Pemphigus

Question 38

If looking at am immunofluoresnce image for pemphigus what would you expect to see?

Answer 38

Basket weave appearance