ORAL MED vesiculobullous conditions

Question 1

Describe the term Bulla?

Answer 1

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

Question 2

describe the term vesicle?

Answer 2

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

Question 3

how do bulla, vesicles become erosion/ ulceration?

Answer 3

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

Question 4

how can we classify vesiculobullous conditions?

Answer 4

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)

Question 5

what is the general cause of vesiculobullous conditions?

Answer 5

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

Question 6

list intraepithelial vesiculobullous conditions?

Answer 6

pemphigus vulgaris
pemphigus foliaceous
paraneoplastic pemphigus
drug induced pemphigus

Question 7

list viral infections which can cause intraepithelial vesiculobullous conditions

Answer 7

herpes simplex
herpes zoster
coxsackie infections

Question 8

list the types of sub-epithelial vesiculobullous conditions?

Answer 8

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

Question 9

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

Answer 9

acantholytic
non-acantholytic

depending on the underlying mechanism responsible for epithelial cell separation

Question 10

describe the term acantholysis?

Answer 10

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

Question 11

what are the acantholytic intraepithelial vesiculobullous conditions?

Answer 11

pemphigus vulgaris
pemphigus foliaceous
paraneoplastic pemphigus
drug induced pemphigus

Question 12

what are the non acantholytic intraepithelial vesiculobullous conditions?

Answer 12

viral infections
- herpes simplex
- herpes zoster
- coxsackie infections

Question 13

pemphigus vulgaris aetiology and epidemiology?

Answer 13

acantholytic intraepithelial

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

Question 14

pemphigus vulgaris signs and symptoms and clinical features?

Answer 14

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

Question 15

what is nikolskys sign?

Answer 15

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

Question 16

list other site involvement of pemphigus vulargis?

Answer 16

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

Question 17

what investigations are performed for pemphigus vulgaris?

Answer 17

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

Question 18

pemphigus vulgaris management?

Answer 18

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

Question 19

name a barrier mouthwash used to locally manage pemphigus vulgaris?

Answer 19

Gelclair

Question 20

give examples of disease modifying anti rheumatic drugs?

Answer 20

azathioprine
mycophenolate mofetil

Question 21

what is the most common case of pemphigus?

Answer 21

pemphigus vulgaris

Question 22

what is paraneoplastic pemphigus most commonly linked to?

Answer 22

lymphoproliferative disorders e.g., lymphoma

Question 23

where do you commonly find pemphigus foliaceous?

Answer 23

skin

Question 24

antibodies of pemphigus foliaceous are directed against?

Answer 24

desmoglein 1

Question 25

how does drug induced pemphigus arise?

Answer 25

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

Question 26

aetiology and epidemiology of mucous membrane pemphigoid?

Answer 26

acantholytic subepithelial aetiology - autoimmune epidemiology - pts over 50, peak 70

Question 27

mucous membrane pemphigoid signs and symptoms, and clinical features?

Answer 27

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

Question 28

other site involvement of mucous membrane pemphigoid?

Answer 28

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

Question 29

why is conjunctival involvement of mucous membrane pemphigoid of significance?

Answer 29

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

Question 30

investigations for mucous membrane pemphigoid?

Answer 30

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

Question 31

mucous membrane pemphigoid management?

Answer 31

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)

Question 32

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

Answer 32

potent: - fluocinolone, betamethasone valerate super potent: - clobetosol

Question 33

what does erythema multiforme affect?

Answer 33

intra/ subepithelial minor - skin plus mild oral major - skin plus other mucosal

Question 34

what triggers erythema multiforme?

Answer 34

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

Question 35

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

Answer 35

primary herpetic gingivostomatitis

Question 36

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

Answer 36

mucous membrane pemphigoid

Question 37

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

Answer 37

pemphigus vulgaris

Question 38

describe the difference in bullae of PV and MMP?

Answer 38

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

Question 39

what drug is used for HSV?

Answer 39

aciclovir

Question 40

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

Answer 40

oral mucosal involvement only

Question 41

when would you use systemic treatment for MMP, PV?

Answer 41

multiple site involvement