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Module 7 > ORAL MED vesiculobullous conditions > Flashcards

ORAL MED vesiculobullous conditions Flashcards

1
Q

Describe the term Bulla?

A

a blister, more than 1cm in diameter that contains clear, serous, or haemorrhagic fluid

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2
Q

describe the term vesicle?

A

a small blister, less than 1cm in diameter that contains clear, serous, or haemorrhagic liquid

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3
Q

how do bulla, vesicles become erosion/ ulceration?

A

rupture of the bulla/ vesicle results in development of erosion/ ulceration

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4
Q

how can we classify vesiculobullous conditions?

A

classified into 2 broad groups depending on the level at which separation occurs:
1. intra-epithelial/ epidermal (within the epithelium/ epidermis)
2. sub-epithelial/ epidermal (below the basement membrane)

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5
Q

what is the general cause of vesiculobullous conditions?

A

they are a result of production of antibodies against components of the epithelium or basement membrane zone

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6
Q

list intraepithelial vesiculobullous conditions?

A

pemphigus vulgaris
pemphigus foliaceous
paraneoplastic pemphigus
drug induced pemphigus

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7
Q

list viral infections which can cause intraepithelial vesiculobullous conditions

A

herpes simplex
herpes zoster
coxsackie infections

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8
Q

list the types of sub-epithelial vesiculobullous conditions?

A

erythema multiforme
pemphigoid
- mucous membrane pemphigoid
- bullous pemphigoid
dermatitis herpetiformis
linear IgA disease
epidermolysis bullosa
epidermolysis acquisita
angina bullosa haemorrhagica
bullous lichen planus

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9
Q

what can intra-epithelial vesiculobullous conditions be further divided into?

A

acantholytic
non-acantholytic

depending on the underlying mechanism responsible for epithelial cell separation

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10
Q

describe the term acantholysis?

A

the separation of keratinocytes within the epidermis due to the loss of adhesion between keratinocytes. It occurs due to the failure of the integrity of the intracellular/ intraepidermal cell junctions

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11
Q

what are the acantholytic intraepithelial vesiculobullous conditions?

A

pemphigus vulgaris
pemphigus foliaceous
paraneoplastic pemphigus
drug induced pemphigus

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12
Q

what are the non acantholytic intraepithelial vesiculobullous conditions?

A

viral infections
- herpes simplex
- herpes zoster
- coxsackie infections

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13
Q

pemphigus vulgaris aetiology and epidemiology?

A

acantholytic intraepithelial

aetiology - autoimmune
epidemiology - rare, HLA linked (south asian)

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14
Q

pemphigus vulgaris signs and symptoms and clinical features?

A

acantholytic intraepithelial

signs and symptoms:
pt may not be aware of oral bullae/ vesicle as they rupture easily
oral mucosal pain as a result of ulceration could limit function
external surface of lips may be involved

clinical features:
flaccis bullae/ vesicles which rupture easily to leave areas of erosion/ ulceration
all areas of oral mucosa may be affected
Nikolskys signs

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15
Q

what is nikolskys sign?

A

a skin finding in which the top layers of the skin slip away from the lower layers when rubbed
present in pemphigus vulgaris

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16
Q

list other site involvement of pemphigus vulargis?

A

pharynx, larynx, nasal, genital, conjuctive, oesophagus, anus and skin

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17
Q

what investigations are performed for pemphigus vulgaris?

A

direct immunofluorescence microscopy (gold standard) to detect autoantibodies:
- desmoglein 3
- desmoglein 1 (only in some pts)

2 biopsies taken:
1. lesional tissue
2. normal tissue

18
Q

pemphigus vulgaris management?

A

recognition by primary care practitioner of significant signs and symptoms even if uncertain of diagnosis
prompt referral to secondary care
management linked to disease severity
involvement of other specialists is key - derm

local: topical analgesics/ barrier mouthwash or topical steroids

systemic:
prednisolone typical starting dose 1mg/kg
steroid sparing disease modifying anti-rheumatic drugs (azathioprine, mycophenolate mofetil)
rituximab - monoclonal antibody against the B lymphocyte surface antigen CD20

19
Q

name a barrier mouthwash used to locally manage pemphigus vulgaris?

A

Gelclair

20
Q

give examples of disease modifying anti rheumatic drugs?

A

azathioprine
mycophenolate mofetil

21
Q

what is the most common case of pemphigus?

A

pemphigus vulgaris

22
Q

what is paraneoplastic pemphigus most commonly linked to?

A

lymphoproliferative disorders e.g., lymphoma

23
Q

where do you commonly find pemphigus foliaceous?

A

skin

24
Q

antibodies of pemphigus foliaceous are directed against?

A

desmoglein 1

25
Q

how does drug induced pemphigus arise?

A

adverse reaction to ACE inhibitors (such as captopril)
may arise as long as 6 months after commencement of medication responsible

26
Q

aetiology and epidemiology of mucous membrane pemphigoid?

A

acantholytic subepithelial

aetiology - autoimmune
epidemiology - pts over 50, peak 70

27
Q

mucous membrane pemphigoid signs and symptoms, and clinical features?

A

acantholytic subepithelial

signs and symptoms:
pt may give history of oral bullae/ vesicles as they may remain intact
oral mucosal pain as a result of ulceration which may limit function

clinical features:
turgid (tense) bullae/ vesicles which rupture to leave areas of erosion and ulceration
all areas of oral mucosa may be affected
may be confined to gingivae and present as desquamative gingivitis

28
Q

other site involvement of mucous membrane pemphigoid?

A

conjunctival
nasal
genital
larynx
oesophagus
skin in 25-30% pts

29
Q

why is conjunctival involvement of mucous membrane pemphigoid of significance?

A

results in scarring (cicatrical pemphigoid) which may lead to loss of sight

30
Q

investigations for mucous membrane pemphigoid?

A

biopsy:
- lesional tissue
- peri-lesional (normal tissue)
usually IgG and/or IgA along basement membrane

IDIF and ELISA also helpful but may be false negatives due to low conc of autoantibodies

31
Q

mucous membrane pemphigoid management?

A

matched to disease severity:
topical analgesic/ barrier mouthwash
topical steroids - can be used in vacuum formed splints to apply potent/ super potent to the gingivae
good OHI critical

systemic:
mild - doxycycline
severe - dapsone, prednisolone, DMARDS (azathioprine, mycophenolate mofetil, rituximab)

32
Q

when managing mucous membrane pemphigoid, what topical steroids are used?

A

potent:
- fluocinolone, betamethasone valerate

super potent:
- clobetosol

33
Q

what does erythema multiforme affect?

A

intra/ subepithelial

minor - skin plus mild oral
major - skin plus other mucosal

34
Q

what triggers erythema multiforme?

A

HSV 70% cases
mycoplasma pneumoniae
medication - anti-convulsants
SJS 10% of pts body

35
Q

what is most likely the diagnosis of a single episode vesiculobullous disease?

A

primary herpetic gingivostomatitis

36
Q

what is most likely the diagnosis of recurrent or persistent vesiculobullous disease?

A

mucous membrane pemphigoid

37
Q

what is most likely the diagnosis of a vesiculobullous disease that affects the skin?

A

pemphigus vulgaris

38
Q

describe the difference in bullae of PV and MMP?

A

PV - fragile, flacid (intraepithelial)
MMP - turgid, more robust (subepithelial)

39
Q

what drug is used for HSV?

A

aciclovir

40
Q

when would you just use topical steroids to treat MMP and PV?

A

oral mucosal involvement only

41
Q

when would you use systemic treatment for MMP, PV?

A

multiple site involvement

Module 7 (58 decks)

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