Vesiculobullous disorders of the oral cavity Flashcards

1
Q

vesicle - define

A

<5mm visible accumulation of fluid within or beneath epithelium
- e.g. a small blister

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2
Q

bullae - define

A

> 5mm visible accumulation of fluid within or beneath epithelium
- e.g. a bigger blister

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3
Q

Give examples of vesiculobullous conditions

A

mucous membrane pemphigoid
pemphigus vulgaris
Erythema multiform
Stevens-Johnson syndorme/ toxic epidermal necrosis

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4
Q

features of normal oral mucosa

A

epithelium (keratinocytes)
- stratified squamous
basement membrane
- non-cellular interface
lamina propria
- collagen, fibroblasts, nerves, blood vessels etc

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5
Q

desmosomes - define

A

protein complexes that join keratinocytes to kertainocytes

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6
Q

function of hema-desmosomes

A

joins basal keratinocytes to basement membrane

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7
Q

mucous membrane pemphigoid - features

A

autoimmune process
50-60 year olds
1:2 male to female ratio

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8
Q

mucous membrane pemphigoid - clinical features

A

oral vesicles/blisters
- ulcers
- robust blisters, sometimes blood filled
heals with scarring
desquamative gingivitis
ocular lesions
- scarring of conductive
anogenital lesions
skin lesions
- scalp
nasal mucosa affected

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9
Q

mucous membrane pemphigoid aetiology

A

unknown cause
likely genetic predisposition
autoimmune

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10
Q

mucous membrane pemphigoid pathogenies

A

antibody (IgG) targeting the basement membrane zone (hemidesmosomes)
complement activation
sub epithelial splitting
= vesicles, blisters, ulcers

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11
Q

mucous membrane pemphigoid diagnosis

A

clinical/histological/immunopathological

biopsy
- H & E staining from affected tissue
- direct immunofluorescence microscopy - from pirilesional tissue
indirect immunofluorescence
- blood sample

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12
Q

MMP direct immunofluorescence - steps

A

tissue biopsy put onto slide and processed
specific antibodies applied to tissue
examined with UV microscope
fluorescence at areas of binding

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13
Q

MMP indirect immunofluorescence 0 steps

A

blood sample taken
- containing primary disease antibody)
- incubated with normal mucosa
- monkey oesophagus
- addition of secondary antibody and fluorophore
- tissue examined
- will show in tissue if antibodies present in serum

less sensitive in MMP
- Will show in 50-80%

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14
Q

MMP management

A

MDT approach
- oral med
- ophthalmology
- gynaecology
- dermatology
low risk
- oral disease only
high risk
- multi-site

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15
Q

pemphigus vulgarise features

A

autoimmune
more common in females

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16
Q

pemphigus vulgaris clinical features

A

blisters, erosions and ulcers
- oral bullae - quickly rupture to leave erosions/ulcers (thin walled blisters that rupture easily)
- heal without scarring
- desquamative gingivitis
- ocular involvement
- aeorodigestive tract
- anogenital blistering
- skin affected
pain
potentially lethal
systemically unwell
- impaired oral intake
- sepsis- secondary infection

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17
Q

pemphigus vulgaris aetiology

A

autoimmune overlap

18
Q

pemphigus vulgaris pathogenesis

A

antibodies (mainly IgG) directed against desmosomes
loss of cell-cell contact in epithelium
- intra-epithelial split forms
- flaccid blisters

19
Q

pemphigus vulgaris - how does pathogenis differ from mucous membrane pemphigoid?

A

MMP hemidesmosomes attacked
- compared with desmosomes in pemphigus vulgaris
MMP sub-epithelial split
- Pemphigus-vulgaris = intra-epithelial split

20
Q

pemphigus vulgaris - diagnosis (clinical/histological/immunopathological)

A

Nikolsky’s sign
- rubbing the mucosa induces a bulla
biopsy
- H and e staining from affected tissue
- direct immunofluorescence microscopy form perilesional tissue
indirect immunofluorescence
- blood sample
- more sensitive in pemphigus vulgaris than mucous membrane pemphigoid

21
Q

Management of pemphigus vulgarise

A

MDT approach
- Iv fluid rescucitation
- feeding
- management of secondary infection
- analgesia

22
Q

PV - mian phase sof management

A

induction of remission
maintenance

23
Q

pemphigus vulgaris - induction of remission steps

A

prednisolone +/- bone protection, gastric protection
commence second agent e.g. rituximab

24
Q

pemphigus vulgaris tx - maintenance steps

A

gradual withdrawal from oral steroids
- aim >10mg prednisolone e
second agent
regular monitoring
topical steroids
benzydamine
excellent oral hygiene

25
Q

paraneoplastic pemphigus features

A

rare variation of pemphigus vulgaris
associated with underlying malignancy
- usually haematological
severe mucosal and skin involvement
typically systemically unwell
high mortality

26
Q

Erythema multiforme features

A

acute onset
hypersensitivity reaction
- can often identify trigger
ulceration and blistering of oral mucosa and lips
skin rash
younger cohort - 10-40 years olds
spectrum of severity
- minor
- major
up to 25% recurrence

27
Q

Erythema multiforme clinical features

A

flu-like prodrome 1-2 weeks
skin lesions, itch
lip blisters, ulceration and crusting
oral ulcers
- particularly anteriorly
eyes, genitals may be effected

28
Q

minor vs major erythema multiforme

A

minor = oral+/- skin targets
major = oral, skin + other mucosa site

29
Q

erythema multiforme aetiology

A

hypersensitivity
- infective = HSV-1 in 15-20%
- drugs - allopurinol, carbamazepine, NSAID’S, phenytoin
- following BCG or hep B immunisations

30
Q

erythema multiforme pathogenisis

A

release of cytokines from CD4 cells
amplified immune response
CD8 and T cell attack keratinocytes
apoptosis and necrosis of keratinocytes

31
Q

diagnosis of erythema multiforme (clinical/histological/immonopathological)

A

clinical history
- triggering agent
biopsy of periolesional tissue
- histology = lymphocytic infiltrate, keratinocyte necrosis, intra and see-epithelial splitting
immunofluorescence - non-specific
HSV serology

32
Q

Erythema multiforme management

A

MDP approach
- feeding fluids
- analgesia
stop any obvious precipitating medication/medication linked to trigger
topical steroids for oral lesions (minor EM)
- systemic steroids for more severe disease
adjunctive oral care
- OHI, CHX, comfort measures
antihistamines for itchy skin

33
Q

recurrent erythema multiforme management

A

consider immunosuppression
- risk/benefit
prophylactic acyclovir
- due to HSV implication

34
Q

Stevens-johnson syndrome/toxic epidermal necrolysis (TEN) features

A

rare
very severe - critical care admission
widespread blistering of skin and oral, pharyngeal, genital, nasal and conjunctival involvement
a spectrum

35
Q

SJS/Toxic epidermal necrocylis aetiology

A

most cases = hypersensitivity to medications
= allopurinol, carbamazepine, NSAIDs, phonotolin, penicillins >20%)
genetic predisposition
100x more common in people with HIV

36
Q

SJS/TEN pathogenesis

A

antigens (medication) presented to T lymphocytes
dysregulated immune reaction
CD8 cells, macrophages and neutrophils into epithelium
release of granulysin
apoptosis and necrosis of keratinocytes
breach of skin/mucosa - ‘skin failure’

37
Q

SJS/TEN diagnosis

A

usually clinical history and examination
skin biopsy = epidermal necrolysis, detachment from dermis

38
Q

SJS/TEN management

A

urgent assessment in specialist acre
- burns unit, intensive therapy unit
A- E approach
- airway
- breathing
- circulation
- sedation/pain management
- temperature management
MDT approach
- dermalology, ophthalmology, ENT, burns team
- CHX, oral lubricants, topical steroids
- skin/wound management
- eye care

39
Q

Linear IgA disease features

A

rare in adults
- more common in children
blistering condition
triggered by medications
clinical features similar to MMP
- blisters, ulcers, erosions of skin and mucosa

40
Q

linear IgA disease management

A

removal of any triggers
MDT approach
topical steroids - mouthwash, ointment
second line - dapsone

41
Q

epidermolysis bulls Acquisita features and management

A

rare
middle aged patient
autoimmune
IgG antibodies target collagen in basement membrane - destabilises epithelial/connective tissue junction
blister formation, erosions, ulceration, scarring from friction/trauma
management
- MDT approach
- topical steroids - mouthwash, ointment
- systemic immunosuppression e.g. prednisolone

42
Q

desquamative gingivitis - summarise

A

clinically descriptive term, not a diagnosis
- desquamation of epithelium causing subsequent erosion of the attached and marginal gingivae
discomfort
treat with topical steroids