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Oral Surgery > Vesiculobullous disorders > Flashcards

Vesiculobullous disorders Flashcards

1
Q

What type of disorder is a vesiculo-bullous disorder?

A

chronic inflammatory disorders

they are unpleasant, chronic, sometimes life-threatening disorders

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2
Q

Mechanism

A

auto-antibodies target structural proteins of desmosome and hemi-desmosomal (adhesion molecules) plaques in skin and mucosa -> leading to formation of blisters: vesicles (small) or bullae (big)

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3
Q

Where are the blisters located?

A

either intra-epithelial (within structure of epithelium) or subepithelial (at junction of epithelium and connective tissue)

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4
Q

Clinical presentations (4)

A

erythema
blisters
erosions
ulcers

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5
Q

Clinical symptoms (3)

A
  • pain at site
  • dysphagia (if in oropharynx)
  • foetor (unpleasant smell of the infection)
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6
Q

2 vesiculobullous disorders most commonly associated with oral disorders/presentations

A

pemphigus vulguris
mucous membrane pemphigoid (most common VB disorder)

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7
Q

Characteristic feature of mucous membrane pemphigoid following the process of erythema, ulceration, erosion etc?

A

scar formation
(can be in oesophagus, larynx, conjunctiva)

Oesophageal Strictures, Laryngeal Stenosis, Conjunctival Cicatrization

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8
Q

How severe are the skin effects of MMP?

A

mild (relative to other conditions like PV as it is a mucous membrane disorder)

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9
Q

Aetiopathogenesis of MMP.
What is the histopathological presentation?

A
  • IgG and/or IgA auto-antobodies target hemi-desmosomes in epithelial basement membrane zone (EMBZ)
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10
Q

What is a subtype of MMP

A

Linear IgA disease (LAD)

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11
Q

What age is MMP most common in?

A

55-65

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12
Q

Describe clinical presentation of MMP

A
  • thick-walled bullous lesion
  • lasts several days
  • irregular erosions/ulcers after bullae burst
  • on NON-keratinised mucosa
  • may be blood filled
  • oral lesions usually heal without scarring
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13
Q

What associated condition may you see with MMP. Describe it (3)

A

desquamative gingivitis

erythematous and hyperaemic mucosa
small bullae formation
extends BEYOND marginal gingiva

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14
Q

How do you diagnose MMP?

A 
- 2 x incisional biopsies 
for histopathology (standard saline) AND immunofluorescence (frozen) technique

-serum

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15
Q

What kind of incisional biopsy if you suspect MMP and why?

A

perilesional - take it of the mucosa surrounding the lesion

-not of the bullae/ulcer as you wont see the process of epithelial detachment

→ want to see the where epithalium detached from lamina propia

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16
Q

What does immunofluorescence do?

A

designed to identify and confirm the appearance seen in histopathology (can see light from fluorophore) Fluorescently-labelled Antibodies to identify bound Auto-Antibodies

  • → see linear IgG along epithelial basement membrane zone (but rmb theres also IgA subtype)*
    direct: biopsy sample
    indirect: patients serum
17
Q

What to do if see patient with MMP?

A

refer to specialist

18
Q

Treatment for MMP (4)

A

topical corticosteroids

systemic corticosteroids

immunosuppressant therapy e.g. azathioprine, dapsone

good OHI

19
Q

Why is Pemphigus Vulgaris potentially lethal?

A

blistering and ulcerations on skin - risk of:

  • infection
  • fluid and protein loss
  • dysphagia
20
Q

First sign of PV

A

oral lesions usually precede skin lesions

21
Q

Describe PV general features

A
  • rare
  • chronic
  • potentially lethal
  • can be drug induced
22
Q

Aetiopathogenesis of PV. Where does it occur?

A

IgG auto-antibody binding targeting Desmosomal proteins - interferes with cell adhesion

intra-epithelial (whereas MMP is subepithelial)

does have acantholysis (whereas MMP doesnt)

23
Q

Clinical features of PV

A
  1. painful, fragile, fluid filled blisters
  2. both skin and oral mucosa but oral lesions precedes
  3. burst within a few hours, shallow ulcers and erosions lesion

(MMP is whole epithelium lining, these are just skin so burst easily)

  1. can be located on soft/hard palate, buccal mucosa, lips, gingiva (not just on non-keratinised mucosa unlike MMP)
  2. can be drug induced
24
Q

Histopathology of MMP vs VB

A

MMP

  • subepithelial bullae
  • no acantholytic cells
  • epithelium detaches from underlying lamina propia( Loss of Connection between Basal Epithelial Cells & Dermis)

VB

  • intra-epithelial bullae (disintegration within epithelium)
  • acantholytic cells (cells coming apart)
  • leucocyte infiltration in lamina propia
25
Q

How do you diagnose suspected PV?

A

incisional biopsy - normal histopath and immunofluorescence

→ also needs to be perilesional

26
Q

What do the different patterns (n-serrated, u-serrated etc) in MMP immunofluorescence samples indicate?

A

precise type of disorder the patient has

27
Q

What is seen in IF of PV vs MMP

A

in direct IF. see Ig (IgG, IgM and C3) glowing and bound to intercellular areas of epithelium (unlike along epithelial basement membrane zone in MMP - IgG and C3, not IgM)

28
Q

What do you for pt with PV?

A

refer to specialist

29
Q

Management of PV

A
  • systemic corticosteroids (Prednisolone 40-60mg daily - maintenance dose)
  • immunosuppressive therapy - Azathioprine, Cyclosporine
  • topical corticosteroids - Betnesol mouthwash (supplemental only, not first line as so severe)
  • maintenance of OH
30
Q

Dx process if you see a VB disorder

A
  • clinical features
  • DDx
  • 2 biopsy samples -histopathology, direct IMF microscopy
  • serum may be taken for immunoserology tests- indirect IMF, ELISA (antibody tests)
  • may consider other microbiological tests
31
Q

Other VB disorders (4)

A
  • erythema multiforme
  • angina bullosa haemorrhagica
  • epidermolysis bullosa (EBA)
  • paraneoplastic pemphigus (in pt with haematological malignancies)
31
Q

Other VB disorders (4)

A
  • erythema multiforme
  • angina bullosa haemorrhagica (blood blister - more common than PV and MMP)
  • epidermolysis bullosa (EBA)
  • paraneoplastic pemphigus (in pt with haematological malignancies)
32
Q

What is Erythema Multiforme

A
  • oral/labial erythema, blistering, ulceration, blood stained crusting
  • EM Minor - target-like lesions on skin
  • EM Major - cutaneous + 2 mucosal sites (oral, genital or eyes)
  • causes: infectious/drug trigger
  • tx: systemic corticosteroids and supportive measures
  • Stevens-Johnsons Syndrome (drug or post viral) up to 10% body surface area affected
  • Toxic Epidermal Necrolysis >30% body SA affected
33
Q

What is Angio Bullosa Haemorrhagica

A
  • blood blisters
  • not uncommon (will come across more than others)
  • particularly seen on soft palate or oropharynx but sometimes buccal mucosa

(angina translates to strangulation - call angina BH as can sometimes get blood blisters at back of throat and feels like being strangled)

34
Q

Clinical photographs

A
35
Q

Diff Dx of LP, MMP, PV

A

Oral Surgery (14 decks)

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