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FINALS - Oral Medicine > Vesiculobullous and Immune Disease > Flashcards

Vesiculobullous and Immune Disease Flashcards

1
Q

What are the two main types of immune mediated disease?

A

Hypersensitivity
- Types 1-5

Immunogenic
- Cell mediated
- Anti-body mediated

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2
Q

Which local immunological diseases manifest within the oral cavity?

A

Aphthous ulcers
Lichen planus
Orofacial granulomatosis

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3
Q

Which systemic immunological diseases can manifest themselves within the oral cavity?

A

Erythema multiforme
Pemphigus
Pemphigoid
Lupus erthematosis
Systemic sclerosis
Sjorgen’s syndrome

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4
Q

What is erythema multiforme?

A

A systemic spectrum disorder resulting from a type 3 hypersensitivity reaction.

Skin and mucosal ulceration and variable orofacial involvement.

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5
Q

What are hypersensitivity diseases?

A

Normal immune mechanisms within the body reacting against innocuous antigens.

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6
Q

What are immunogenic diseases?

A

An abnormal response of the immune system leading to a disease process. Can be cell or antibody mediated.

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7
Q

Give three examples of diseases resulting from cell mediated immunity?

A

Aphthous ulcers
Lichen planus
Orofacial granulomatosis

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8
Q

Give two examples of diseases resulting from antibody mediated immunity?

A

Pemphigus
Pemphigoid

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9
Q

What is pemphigus?

A

Pemphigus is a disease that causes blistering of the skin and the inside of the mouth, nose, throat, eyes, and genitals

Results from antibody mediated autoimmune disease.

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10
Q

What is pemphigoid?

A

A rare skin condition that causes large, fluid-filled blisters.

Results from antibody mediated autoimmune disease.

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11
Q

What is the mechanism by which immunological disease can cause breakdown of the skin?

A

Auto-antibody attack on skin components causing loss of cell-cell adhesion

Split’ forms in skin
Fills with inflammatory exudate
Forms vesicle/blister

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12
Q

What two medications can be used for treatment of erythema multiforme?

A

Systemic steroids
Systemic aciclovir

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13
Q

What is angina bullosa haemorrhagica?

A

Oral blood blisters lasting one hour before bursting.

Relatively painless and possibly caused by minor trauma.

Heal with no scarring within days.

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14
Q

What medications are available for pemphigoid?

A

Steroids
Immune modulating drugs

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15
Q

What is the difference between epitopes and antigens?

A
  • Antigens are the big immunological site in which antibody bind to it
  • Epitope is the specific part of the antigen to which the antibody binds
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16
Q

Immunologically what happens during skin disease?

A
  • Auto-antibody attack on skin components causing loss of cell-cell adhesion
  • Causes loss of desmosomes to hemidesmosome by desmoglein attachment allowing cell layers to split
  • Split forms in the skin
  • Fills with inflammatory exudate
  • Forms vesicle/blister
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17
Q

What is the difference between vesicle and blister?

A
  • Vesicles 1-2mm
  • Blister largers
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18
Q

What test is the most useful when suspected immuno bolus condition?

A
  • Direct immunoflurorescnce
  • Antibody mediated tissue disease
  • Antibody causing disease targeted as it is bound to tissue, antigen containing fluorescein that binds to antibody
  • If present then fluorescien shows up
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19
Q

What is indirect immunofluorescence ?

A
  • Circulating antibody not yet bound to tissue
  • Detected by immunofluorescnce from plasma sample
  • Not always useful for diagnosis but good for monitoring disease activity
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20
Q

What is erythema multiforme?

A
  • Spectrum disorder of immunogenic related skin and mucosa ulceration with variable orofacial involvement
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21
Q

What is the onset for erythema multiforme? What response does it create?

A
  • Acute onset due to antigens being presented have been met before therefore antibodies rapidly produced from memory B cells
  • Antigens and antibodies bind forming a complex in circulation and now unable to pass through capillaries
  • When the large Complex gets to the tissues it becomes wedged and activates complements within the blood vessels causing a perivascular response
  • Blistering or ulceration of the tissues
22
Q

Where on the body can lesions of erythema multiforme be shown?

A
  • Skin
  • Genital
  • Oral
  • Oropharyngeal
  • Combo of these
  • Called a spectrum disorder
23
Q

What is the term given to the most extreme form of erythema multiform? Where does it present on the body?

A
  • Stevens-Johnsons syndrome
  • Severe multisystem involvement
  • Skin, conjunctivae, nose, pharynx, mouth, genital
24
Q

In oral erythema multiforme what mucosa can be involved?

A
  • All types of mucosa including keratinsed due to the lesion affecting capillaries
25
Q

What are the lesions of oral erythema multiforme similar to?

A
  • Primary herpetic gingivostomatitis
26
Q

What is the aetiology of oral erythema multiform?

A
  • Reactivation of herpes simplex virus triggers the immune reaction leading to this type 3 hypersensitivty (can give aciclovir prophylactically)
    -Mycoplasma
27
Q

What age group is most commonly affected by erythema multiforme?

A
  • Young males in early 20s
  • Have periods of ulceration for 2-3weeks which are very painful
28
Q

What is the urgent txt for erythema multiforme?

A
  • High dose prednisolone (up to 60mg/day) to suppress the antibody-antigen reaction and the complement activation within the tissue
  • Systemic aciclovir can be given but only 2nd to systemic steroid as above
  • Keep hydration high (sometimes mouth and pharynx so painful to let fluids into, end up in hospital due to dehydration)
  • encourage Analgesia as required
29
Q

What lesion do these images show?

A
  • Erythema multiform
  • Target lesions on the skin with central wheel and perilesional area of pale the erythematous tissues
  • Crusting of the lips due to ulceration and healing of lesions on oral mucosa
  • in the mouth shown Coalescent areas of ulceration more prominent towards front of mouth but can present further back and on pharynx
30
Q

What is the txt for recurrent problems of erythema mulitforme?

A
  • Consider prophylactic aciclovir daily
  • Can perform allergy test to test for a wide variety of environmental triggers - particularly mycoplasma
31
Q

What is Angina Bullosa Haemorrhagica?

A

Meaning tight blister bloodfilled

  • Commonest oral blistering condition
  • Blood blisters in the mouth
32
Q

What are the most common sites for Angina Bullosa Haemorrhagica (ABH)? What is the onset? Is it painful?

A
  • Buccal mucosa and soft palate common sites
  • Can present on hard palate and these can be uncomfortable as feels swallowing is impacted
  • Rapid onset of a few mins, last about 1 hr and then burst
  • Relatively painless and heal with no scarring within days
  • Poss due to minor trauma
33
Q

What is the cause of ABH? Does it have specific aetiology?

A
  • No aetiology
  • Has non specific ulceration
  • DIF and IIF negative
  • No platelet/coagulation defect
  • Unsure of cause
34
Q

What is the txt of ABH?

A
  • As we do not know what causes it or why it appears the way it does
  • txt symptomatic relief
  • Chlorhexidine mouthwash or diflam spray until blister subsides
  • May recur
  • Reasure pt it is benign
  • Triggers might be eating or steroidal inhalers, do not stop eating or using the inhaler
35
Q

What are these lesions called?

A
  • Angina bullosa Haemorrhagica
36
Q

What is Pemphigoid? What are the characteristics of a lesion?

A
  • Relatively common immunobullous disease that is SUBEPITHELIAL antibody attack
  • Antibodies cause seperation of the epithelium at the basement membrane from connective tissue
  • Full thickness of epithelium is released therefore they are thick walled blisters
  • Usually persist
  • Can appear clear or blood filled blisters
37
Q

What are the 3 different forms and presentations of Pemphigoid?

A

Bullous Pemphigoid when affecting Skin (can develop oral lesions)

Mucous Membrane Pemphigoid when affects all mucous membranes including eye, genital, oral

Cicatricial Pemphigoid is a subset of Mucous membrane pemphigoid when affects mucosal but has scarring

38
Q

What type of biopsy sample would you take for a suspected Pemphigoid lesion?

A
  • When taking a biopsy take from perilesional region not just the area of blister itself as this may lead to no epithelium being found so diagnosis difficult
39
Q

What is the histopathology of Pemphigoid lesions? (Role of hemidesmosome)

A
  • Hemi desmosomes attach to the epithelium through basement membrane to connective tissue
  • Attack of circulating antibodies attack these hemi desmosomes therefore allowing the loss of attachment of epithelium to connective tissue (termed a sub epithelial split at epithelial/connective tissue junction)
40
Q

What lesion is this showing? Describe what you see in both images

A
  • Pemphigoid lesion

Left image
- Sub epithelial split at epithelial/connective tissue junction due to hemi desmosomes attachment
- Can see lots of inflammatory exudate (fluid) below the split which is present in the blister

Right image
- Can see the subepithelial split which is stops around halfway across the epithelium
- Can’t see any inflammatory exudate
- Different stages of blister formation

41
Q

What is the disease creating these lesions? Describe the lesions and the issues they may provide for the pt

A
  • Bullous Pemphigoid
    Right
  • Blood stained filled intact bullous lesions

Left
- Intact clear fluid filled bulla
- Ruptured bulla showing loss of epithelial barrier
- Can allow infection into the skin
- Can also lead to dehydration due to the weeping ruptured blisters

42
Q

What is the most useful test for a pt with Pemphigoid?

A
  • Direct Immunofluorescence
  • Look at image on right hand side , this shows perilesional biopsy of pemphigoid (epithelial and connective tissue junction is in contact)
  • Can see linear immunofluorescent staining along the epithelial basement membrane
  • Fluorescein tag is attached to antibody which binds to circulating antibody bound to the pemphigoid antigen in basement membrane
  • Where we see the linear staining is where we have pemphigoid pre blister formation
43
Q

What antibody is found in pemphigoid lesions?

A

Most common in standard pemphigoid
- C3 (always seen)
- IgG
Less common
- IgM
-IgA

44
Q

If IgA is found in pemphigoid lesions what are the two disease it may be?

A
  • If linear staining with C3 = Linear IgA disease

If granular IgA and C3 deposits = Dermatitis Herpetiformis

45
Q

Describe this image. What can this cause? What disease is being shown?

A
  • Adhesion of the bulbar and palpebral conjunctiva known as Symblepharon
  • Restrict eye movement causing diplopia
  • Can occur in Mucous membrane Pemphigoid
46
Q

What is the management of Pemphigoid lesions?

A
  • Steroids
  • Immune modulating drugs such as azathioprine and mycophenolate, dapsone
47
Q

What is Pemphigus? What desmosomes are involved and their role?

A
  • Immune mediated antibody directed disease
  • Intraepithelial bullae form due to circulating antibody being formed against the desmosomes joining the epithelial cells to each other, causing the epithelial cells to lose adhesion to each other
  • Very rare to see intact fluid filled blisters due to thinning of the epithelium
  • Present with erosive areas , mucosal surface loss as cell are gradually lost from surface of epithelium due to disease
48
Q

Who is most likely affected in pemphigus and what sites are commonly affected?

A
  • Uncommon before age 50
  • Females > Males
  • Ashkenazi jews genetic predisposition
  • Oral mucosa present first then skin later develops (can be up to 3 years later)
49
Q

Describe the histopathology of Pemphigus

A
  • Loss of epithelium and Shedding of epithelium layer supra basally
  • Tzank cells seen with pemphigus
50
Q

Describe the immunoflurescence of this Pemphigus lesion

A
  • Important to take perilesional tissue
  • Pemphigus antibody is attacking desmosomes present on many of the surface of epithelial cells
  • Shows basket weave pattern of antibody binding around each of the epithelial cells
51
Q

What antibodies are common in Pemphigus Vulgaris?

A
  • C3
  • IgG
52
Q

What disease are these images showing?

A
  • Pemphigus
  • Loss of epithelial covering showing redness

FINALS - Oral Medicine (13 decks)

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