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BDS5 Oral Med > Vesiculobullous Conditions > Flashcards

Vesiculobullous Conditions Flashcards

(48 cards)

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1
Q

What is a Vesicle?

A

a small fluid filled blister <5mm

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2
Q

What is a Bulous?

A

a large fluid filled blister > 5mm

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3
Q

What is the epithelial classification of blistering?

A
  1. Intra-epithelial = fragile blisters
  2. Sub-epithelial = flimsy but long lasting blister
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4
Q

What 5 autoimmune blistering conditions?

A

1) Pemphigus Vulgaris (PV)
2) Mucous Membrane Pemphigoid (MMP)
3) Bullous Pemphigoid
4) Dermatitis Herpetiformis
5) Linear IgA disease

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5
Q

State 2 idiopathic blistering conditions?

A

Erythema Multiforme
Angina Bullosa Haemorrhagica (ABH)

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6
Q

State a collagen defect blistering condition?

A

Epidermolysis Bullosa

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7
Q

State 4 infectious (viral) blistering conditions?

A
  1. HSV
  2. HZV
  3. Hand,foot+ mouth disease (Coxsackie)
  4. Herpangia
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8
Q

What two conditions/types of blistering result in INTRA-EPITHELIAL BLISTERING?

A
  1. Pemphigus Vulgaris
  2. Viral infections
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9
Q

What investigations are used to diagnose blistering conditions?

A
  1. Biopsy - tissue sample (Direct immunofluorescence + histopathology)
  2. Blood Test (Indirect immunofluorescence + blood serum)
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10
Q

What type of investigation is direct immunofluorescence and give details about it?

A

= Biopsy

Method: Incisional biopsy (of lesions + normal skin)

Demonstrates bound immune complexes in mucosa or skin

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11
Q

What would a blood serum sample for blistering conditions show?

A

Conc. of haemotinics/ antibodies

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12
Q

What type of investigation is indirect immunofluorescence and give details about it?

A

= blood test (blood looked at under light)

Detects autoantibodies in serum

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13
Q

What is Pemphigus Vulgarus?

A

= autoimmune disease where IgG autoantibodies destroy desmosomes causing INTRA-EPITHELIAL blistering

(desmosomes are responsible for cell-cell adhesion i.e. it is the glue that holds cells together, igG autoantibody attacks desmosomes resulting in the break down of cell adhesion - thus intraepithelial blistering

we get blisters affecting oral mucosa WEEKS before anywhere else in the body; IO signs 1st)

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14
Q

Who does Pemphigus Vulgaris affect?

A

40-60yrs old
M=F

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15
Q

What is the aetiology (cause) of Pemphigus Vulgaris?

A
  • genetic predisposition
  • diet (e.g. garlic + red wine)
  • Medication:
    1. Sulphydryl drugs i.e. penicillamine
    2. Non-thiol drugs w/ an active amide group i.e. diclofenac
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16
Q

What is the clinical presentation of Pemphigus Vulgarus?

DONE

A

Where?
- keratinised surfaces esp. on junction of hard + soft palate
- hard palate
- dorsum of tongue
- gingiva (+ buccal mucosa)

What?
- Oral bullae
- Nikolsky’s sign (oral blisters rapidly break down to form slow non-healing erosions/ulcer + white patches)
- Desquamative Gingivitis(in 60%)
- Erosive Lips

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17
Q

What investigations are used for diagnosis of Pemphigus Vulgarus?

A
  1. Incisional biopsy of fresh blister+ normal tissue for histology which shows “intra-epithelial clefting + acantholysis”
  2. Direct immunofluorescence (biopsy)
  3. Serum blood test for IgG autoantibody
  4. Indirect immunofluorescence (blood test)
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18
Q

What is the tx for Pemphigus Vulgaris?

A

1) Topical Corticosteroids (betamethasone m/w)

2) Systemic Corticosteroids (prednisolone)

3) Steroid sparing agents (azathioprine + cyclosporine)

4) Immunotherapy:
- IV monoclonal antibodies
- IV immunoglobulins
- Plasmapheresis

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19
Q

What is Mucous Membrane Pemphigoid? (MMP)

A

SUB-EPITHELIAL chronic blistering autoimmune disease

where the body attacks the basement membrane zone + epithelium lifts off underlying connective tissue (CT).

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20
Q

What is the aetiology of Mucous Membrane Pemphigoid? (MMP)

Study These Flashcards
A

unknown aetiology

21
Q

Who does Mucous Membrane Pemphigoid affect?

Study These Flashcards
A

F> M
~ elderly 60yr old

22
Q

What is the oral presentation of Mucous Membrane Pemphigoid?

Study These Flashcards
A

Site:
any oral site
lip lesions = rare

Symptoms:
Large blood filled bullae that ruptures into chronic painful (pseudomembranous) erosions + ulcerated lesions

Desquamative gingivitis (blisters involving attached gingiva)

Nikolsky’s sign

Chronic Soreness

Dysphagia

KEY: may resemble LICHEN PLANUS

23
Q

What is systemic presentation of Mucous Membrane Pemphigoid?

Study These Flashcards
A

Occular involvement
- Visual impairment + blindness
- Chronic conjunctivitis
- one eye then both eyes in 2 years
- scarring then fibrosis

  • vesicles/ ulcerations if very progressive
  • skin (scalp + face + neck)
  • Irreversible alopecia
  • nose
  • larynx + oesophagus
    -genitals
24
Q

What investigations are conducted for the diagnosis of Mucous Membrane Pemphagoid?

Study These Flashcards
A
  1. Biopsy (histology -full thickness of epithelium lifts off CT
  2. Direct immunofluorescence - deposits of IgG/ C3
  3. Occular examination
25
What is the tx for Mucous Membrane Pemphagoid?
1. **Dapsone** 2. Topical corticosteroids (betamethasone m/w) 3. Systemic corticosteroids (prednisolone if ocular involvement) 4. **anti-inflammatory antibiotics (doxycycline)** 5. immunosuppressant (non-steroidal i.e. azathioprine)
26
What is Bullous Pemphigoid?
= sub epithelial autoimmune blistering condition, erythema blisters that develop on skin (rarely mouth) - initially itchy then erosions (scaring not prominent)
27
What is the management for Bullous pemphigoid?
Same investigations + tx as Mucous Membrane Pemphigoid
28
What is Dermatitis Herpetiformis?
= autoimmune **sub-epithelial** blistering disease assoc. w/ celiac disease
29
Who does Dermatitis Herpetiformis affect?
- younger age group - (Irish people)
30
What is the cutaneous presentation of Dermatitis Herpetiformis?
chronic pruritic papulovesicular rash on the buttocks, elbows & knees, appears as small blisters on itchy base
31
What is the oral presentation of Dermatitis Herpetiformis?
transient superficial blisters (+ tender non-specific ulcers)
32
What is the management for Dermatitis Herpetiformis? a) investigations?
1.Histopathology (biopsy) → localised splitting at the basement membrane zone (BMZ) 2. Direct immunofluorescence (biopsy) → granular IgA immunofluorescent deposits involving the BMZ of the dermal papillae 3. Blood serum test → positive for tissue transglutaminase IgA
33
What is the management for Dermatitis Herpetiformis? b) tx?
- Dietary restriction w/ gluten free diet - Dapsone (gel or tablet)
34
What is Linear IgA Disease?
chronic autoimmune sub epithelial blistering mucocutaneous disease - ORAL LESIONS present majority - Ocular lesions infrequent (not assoc/ w coeliac disease)
35
What is the management of Linear IgA disease?
Investigation: - Direct immunofluorescence (biopsy) → linear deposits of IgA at CT junction w/ separation at BMZ Tx: dapsone / corticosteroids
36
What is Erythema Multiforme?
immune- mediated type III mucocutaneous blistering condition, triggered by: 1. Infections (e.g. recurrent HSV, HZV) 2. Drugs (antiretrovirals, antibxs, antifungals, NSAIDS, barbiturates) 3. Systemic e.g. pregnancy, malignancy, SLE 4. Idiopathic
37
Who does Erythema Multiforme affect?
<30 yr old & M>F
38
What is the cutaneous presentation of Erythema Multiforme?
central blister surrounded by oedematous ring with erythematous border = target lesion Where? Skin>palms>soles
39
What is the oral presentation of Erythema Multiforme?
bullae that rapidly break into irregular ulcers, bleed, form crusts (gingiva rarely affected) Lips + palate most affected
40
What is the tx for Erythema Multiforme?
Tx: - Usually self-resolving - Acyclovir 5% topical cream AT BEGINNING STAGE - Penciclovir 1% cream, low compliance due to re-application every 2 hrs - Coffee grounds ? - Consider systemic acyclovir 400mg x2 day if the pt has recurrent HSV
41
What is Angina Bullosa Haemorrhagica? (ABH)
= Chronic idiopathic sub-epithelial condition causing blood blisters
42
Who does Angina Bullosa Haemorrhagica affect?
Elderly Steroid spray users (asthmatics) Diabetics Genetic predisposition
43
What is the oral presentation of Angina Bullosa Haemorrhagica?
- sudden appearance of blood filled blisters (often associated w/ consumption of dry/rough food, pt feels like they're choking) - solitary (alone) - 2-3cm diameter (quite large) -bursts spontaneously ~24hrs, heals in 7-10days w/o scarring - recurs every few years SITE: junction of hard + soft palate
44
What is the management of ABH?
Investigations: - FBC/Coagulation screen Tx: - Benzydamine & chlorhexidine m/w - Incise large intact blood blister (to prevent pt from choking feeling & respiratory embarrassment)
45
What is Epidermolysis Bullosa?
= sub epithelial collagen defect blistering condition who? Any age F>M
46
What are the 3 forms of Epidermolysis Bullosa?
1. EB simplex – least severe 2. EB dystrophica [genetic] – fragile skin & mucosa 3. EB lethalis – lethal/deadly
47
What is the clinical presentation of Epidermolysis Bullosa?
Any trauma to skin or mucosa resulting in separation of underlying CT, then scaring and deformity There OH + eating is problematic
48
What is the management of Epidermolysis Bullosa?
Investigations: - Direct immunofluorescence → linear IgG deposits & collagen type 7 anchoring fibril target antigen Tx: - Oral corticosteroids - Immunosuppressives +/- dapsone

BDS5 Oral Med (12 decks)

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