Vesiculobullous Disease Flashcards

1
Q

What immune reactions can the body produce?

A

Hypersensitivity reactions type 1-5

Immunogenic responses

  • cell mediated immunity
  • antibody mediated immunity
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2
Q

Give some diseases that produce local responses in the oral mucosa

A

Aphthous ulcers

Lichen planus

Orofacial ganulomatosis

(All cell mediated immune responses)

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3
Q

Give some systemic diseases that can present with localised oral mucosa lesions

A

Erythema multiforme (type 3 hypersensitivity)

Pemphigus (antibody mediated)

Pemphigoid (antibody mediated)

Lupus erythematosis

Systemic sclerosis

Sjogrens

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4
Q

Why are common blistering skin lesions often connected to oral blistering?

A

Both oral mucosa and skin share common antigens due to having the same embryonic origin

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5
Q

Difference between vesicle and blister?

A

Size

Vesicle 1-2mm

Blister is larger

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6
Q

How does immunological skin disease result in a blister or vesicle?

A

Auto-antibody attack on skin components causes a loss in cell - cell adhesion

This forms a split in the thin that:
- fills with inflammatory exudate
- forms vesicle / blister

Split caused by attack on protein desmoglein which in turn causes splitting of desmosomes that hold cells together

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7
Q

What does direct and indirect immunofluorescence show

A

Direct = antibodies bound to tissue

Indirect = antibodies not yet bound to tissue and is in plasma

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8
Q

What is angina bullous haemorrhagica?

Describe it

A

‘Blood blisters in the mouth’
- Benign
- rapid onset
- appear in a few minutes and then burst after around an hour
- relatively painless
- significant lesions can appear around vibrating line that can give pt feeling of occlusion of airway

Most common oral blistering condition

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9
Q

How treat angina bullish haemorrhagica?

A

Symptomatic relief

Chlorhexadine mouthwash or difflam spray

Reassure it is benign

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10
Q

What is pemphigoid? What causes it?

A

Thick walled blisters of the full epidermis
- clear or blood filled
- on skin or mucosa

Caused by sub epithelial antibody attack.
- antibodies cause separation of the epithelium at the basement membrane from the connective tissue by attacking hemidesmosomes
- achieves this as IgG and IgA antibodies bind to BP180 protein to form an immune complex. These immune complexes activate the complement system.
- triggers an influx of inflammatory cells and formation of a blister in the area.

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11
Q

What is the best test for pemphigoid?

A

Direct immunofluorescence

  • pemphigoid antibodies bound to the antigen of the basement membrane
  • causing fluorescence
  • C3 and IgG detected
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12
Q

How manage pemphigoid?

A

Steroids

Immune modulating drugs - azathioprine

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13
Q

What is pemphigus?

A

Circulating antibodies IgG attack desmoglein 1 and desmoglein 3 leading to destruction of the desmosomal complex that holds keratinocytes together.

Intra epithelial blisters form
- blisters burst and spread
- variable number of cells above so easily bursts
- results in epithelial / mucosal erosions

Oral lesions precursor to skin lesions by up to 3 years

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14
Q

How tell difference between pemphigoid and pemphigus?

A

Rare to see intact bulla in pemphigus

Intact bulla on ora mucosa = pemphigoid

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15
Q

Test for pemphigus?

A

Immunofluorescence

  • basket weave pattern

Vs linear pattern in pemphigoi

C3 and IgG found again

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16
Q

Tx for pemphigus?

Prognosis?

A

High dose steroids

Fatal without treatment

Now, often complications of treatment are the major cause of death

17
Q

Intra oral manifestations of pemphigoid?

A

Bullae formation - these quickly rupture to leave erosions of the mucosa

Erythema - open sores or shallow wounds

Stricture - scarring - restricted movement or narrowing of oral cavity / airway

18
Q

Extra oral manifestations of MMP?

A

Ocular involvement - inflammation of the eyes - visual impairment

Skin lesions - blistering and erosions of the skin - skin discomfort

Bullae and erosion of other mucous membranes

All can lead to psychosocial effects

19
Q

What is nikolskys sign?

A

Lateral pressure placed on a bullae

Positive sign = the exfoliation of the outermost layer by slight rubbing of the skin

20
Q

How can indirect immunofluorescence / Elisa testing be used to test for MMP?

A

IIF - detects circulating IgG autoantibodies

Elisa testing - tests for BP180 and BP230

21
Q

Histological signs of pemphigus vulgaris?

A

Perilesional biopsy

Acantholysis - loss of cell-cell adhesion in the epithelium

Tzanck cells - individual large round keratinocytes with hypertrophic nucleus

Inflammatory exudate within epithelial layers

22
Q

What is ELISA testing?

A

Enzyme linked immunosorbent assay

23
Q

Epidemiology of MMP and PV?

A

50/60 years old

Slightly more common in females

PV more common in Jewish-American population

24
Q

Basically - What is EM?

A

Spectrum disorder with skin and mucosal ulceration with varying oral involvement

25
Q

Epidemiology of EM?

A

Young adults between 20 and 40 with predominance in males

26
Q

Causes of EM?

A

Type 3 hypersensitivity reaction
- herpes virus immune complexes depositing in tissues leading to an immune reaction

Type 4 hypersensitivity to drug reaction
- T cell mediated inflammatory reaction to a drug such as:

antibiotics,
NSAIDS,
TNF-A inhibitors,
Anticonvulsants

27
Q

Types of EM?

A

Minor - only one site - often oral mucosa

Major - more than 2 mucosal sites

Steven Johnson syndrome - <10% of body affected with widespread skin blistering and scarring

Toxic epidermal necrolysis - >30% of body

28
Q

Mucosal features of EM?

A

Blisters and shallow erosions

White overlying pseudomembrane

29
Q

Cutaneous features of EM?

A

Round Target like lesions that are erythematous with concentric rings

30
Q

Histological presentation of EM?

A

Keratinocyte necrosis

Acanthosis

Elongated rete pegs

31
Q

Tx of EM?

A

Often not necessary if very mild

Identify the aetiology is crucial

If drug reaction:
- stop drug and clear infection before systemic tx
- topical steroids and anaesthetics for oral cavity

If herpes / recurrent
- antiviral therapy with aciclovir
- systemic corticosteroids and topical corticosteroids