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Oral Medicine > Vesiculobullous Disease > Flashcards

Vesiculobullous Disease Flashcards

1
Q

What type of immune reaction is pemphigus and Pemphigoid?

A

Antibody mediated immunity

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2
Q

Why do some skin diseases also affect the mouth/genital area?

A

Skin and oral/genital mucosa share many common antigens and epitopes- so the antibodies attack the skin and the oral mucosa.

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3
Q

What is a vesiculobullous disorder?

A

Disorder which results in blistering- autoimmune disorder.

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4
Q

What processes occur which results in pemphigus and pemphigoid?

A

Auto-antibodies attach specific skin components, which causes loss of cell to cell adhesion.

Split forms in the skin- fills with inflammatory exudate and forms a blister.

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5
Q

What are the specific auto-antibody targets for Pemphigus and Pemphigoid?

A

Pemphigoid- BP180 and BP230
- Hemidesmosomes

Pemphigus- Desmoglein 1 and desmoglein 3
- Desmosomes

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6
Q

Describe the pathogenesis of Pemphigoid and Pemphigus?

A

Presence of immunoglobulin antibodies against proteins on the cell surface of keratinocytes.

Auto-antibodies associated with pemphigoid and pemphigus attack hemidesmosomes/desmosomes.

Complement is activated, initiating the immune response, causing loss of adhesion of the hemidesmosomes/desmosomes.

Cell layers split apart.

Blister formation occurs.

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7
Q

Why are blisters formed in pemphigoid thicker and more likely to still be present intact in the oral cavity?

A

The auto-antibody target for pemphigoid are hemidesmosomes.

Hemisdesmosomes attach epithelial cells to the basement membrane, therefore, the fluid exudate that forms when they split rom each other, it much deeper than in pemphigus. These are harder burst.

Sub-epithelial split.

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8
Q

Why might some blisters in Pemphigoid be filled with blood?

A

Some fluid can come from the connective tissue- there may be some leakage of red blood cells into the blister.

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9
Q

Describe the different forms of Pemphigoid?

A

Bullous Pemphigoid- skin
Mucous Membrane pemphigois- all mucous membranes- eye, genital, oral
Cicatritial Pemphigoid- mucous with scarring

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10
Q

Why is it important to take a peri-lesional biopsy, rather than from the blister itself?

A

If you took it from the blister itself, then there is usually no epithelium left, so there will be no result on histology.

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11
Q

What are some of the signs and symptoms of Pemphigoid that you might see in a patient?

A

Desquamative gingivitis- more likely to have gingival involvement in Pemphigoid.

Erosions, ulcerations, erythema of the oral cavity.
- As a result of the blisters bursting.

Pain and inability to eat foods/drink

Thick walled blisters filled with fluid, which can burst to form exposed connective tissue and inflammatory exudate.

Symblepharon in the eye

Non-specific conjunctivitis

Dryness in the eyes/irritation of the eyes

Genital involvement

Larynx- sore throat, hoarseness, breathing difficulty.

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12
Q

What is Nikolsky’s sign?

A

Skin finding- top layers of the skin slip away from the lower layers when rubbed.

Lateral pressure causes separation of the epidermis from the dermis.

Seen in Pemphigus but not Pemphigoid.

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13
Q

What tests would you request if you suspected Pemphigoid?

A

FBC, also look for auto-antibodies BP180 and BP230.
Incisional biopsy- direct immunofluorescence
Another incisional biopsy for histological testing.

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14
Q

What findings in direct immunofluorescence would suggest pemphigoid?

A

Linear staining along the basement membrane- C3 and IgG, IgA occasionally found.

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15
Q

How does immunofluorescence work?

A

Fluoroscein tag is attached to the antibody which binds to the circulating antibody bound to the antigen bound in the basement membrane,

When it binds, the fluorescein tag will show up as green.

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16
Q

What is symblepharon?

A

Scarring of the mucosa over the eye- binding of the eye surface to the eyelid.

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17
Q

What histological findings would suggest Pemphigoid?

A

H&E staining- Subepidermal split from the basement membrane with fluid infiltrate.

Tissue bound auto-antibodies.

Immune cell infiltration- studies have shown mainly eosinophils.

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18
Q

What findings would you expect to see in indirect immunofluorescence?

A

Circulating autoantibodies for IgG.

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19
Q

What results from ELISA would suggest Pemphigoid?

A

Circulating BP180 and BP230.

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20
Q

What cohort of people are usually affected by Pemphigoid?

A

Females, above 60 years old.

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21
Q

What is Pemphigus?

A

Autoimmune disorder, which results in intraepithelial bullae formation.

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22
Q

What type of patient is most commonly affected by Pemphigus Vulgaris?

A

females, aged 50-60 year old.

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23
Q

Describe the pathogenesis of Pemphigus?

A

Anti-Dsg1 and anti-dsg2 autoantibodies form, which attack the proteins on the cell surface of keratinocytes.

Loss of cell to cell adhesion between keratinocytes- acantholysis.

Dig endocytosis and desmosome disassembly

Intercellular stretch at non-acantholytic cell layer.

Inter-epithelial bullae form

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24
Q

Why is it rare to see intact bullae in Pemphigus?

A

Intra-epithelial bullae are present in pemphigus. These are much closer to the surface of the epidermis and so are more easily traumatised and burst.

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25
Q

What signs and symptoms might a patient present with if they have Pemphigus?

A

Oral erosion- exposed connective tissue after the blister has burst
- mucosa is often the first site and then eventually moves to the skin as well.

May have gingival involvement but more likely for this to happen in pemphigoid.

Could also have nasal, pharyngeal, laryngeal, oesophageal, ano-genital, tracheobronchial involvement.
- Vaginal irritation
- Dysphagia
- Painful sexual intercourse
- Occular irritation
- Painful swallowing/chewing
- Voice hoarseness

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26
Q

What are the histological features of PV?

A

H&E staining of a sample.

Suprabasilar acantholysis
- loss of cell to cell adhesion between epidermal cells.

Tzanck cells- large round keratinocytes with a hypertrophic nucleus and abundant basophilic cytoplasm.

Tombstoning at the basement membrane- retention of basal keratinocytes at the basement membrane.

Inflammation of underlying connective tissue.

27
Q

What are the direct immunofluorescence features seen in Pemphigus?

A

Tests for the presence of autoantibodies in non-affected oral mucosa.

Basket weave appearance- autoantibodies bound by fluorescein binds to IgG and C3 between the epithelial cells.

28
Q

What type of biopsy is required for DIF?

A

Perilesional biopsy of oral mucosa, roughly 4mm from a. vesicle or erosion.

Biopsies taken from the affected oral mucosa are more likely to produce a false negative because of destruction of immuinoreactants during the disease process.

29
Q

What type of sample is required for Indirect immunofluorescence and ELISA testing?

A

Serum sample.

30
Q

What results would you expect for IIF and ELISA if someone had Pemphigus?

A

IIF detects antibodies not yet bound to tissue.
- fish net appearance.

ELISA tests for antibodies against desmoglein 3 +/- desmoglein 1
- anti-dsg1 antibodies- only skin blisters
- anti-dsg3 antibodies- erosions.ulcerations of mucosal membranes,
- Both anti-dsg1 and anti-dsg3- both skin and mucosal lesions.

31
Q

What is the initial therapy used for pemphigus and pemphigoid?

A

Prednisolone

32
Q

What is the mechanism of action of prednisolone?

A

Prednisolone is a glucocorticoid which decreases inflammation via binding to cellular glucocorticoid receptors, which inhibits inflammatory cells and suppresses expression of inflammatory mediators.

Anti-inflammatory, immunosuppressive, antineoplastic and vasoconstrictive effects.

33
Q

What other drugs may be used for Pemphigus/Pemphigoid?

A

Azothioprine
Mycophenolate
Cyclophosphamide

34
Q

What is the mechanism of action of Azothioprine?

A

Downregulates purine metabolism and blocks synthesis of DNA, RNA and proteins.

Reduces activity of Langerhans cells, monocytes, T and B cells.

35
Q

What are the unwanted side effects of Azothioprine?

A

Diarrhoea
Bone marrow suppression
Aphthous stomatitis
Leukopenia
Thrombocytopenia

36
Q

In what group of patients can you not take Azothioprine?

A

Pregnant or breast feeding
- can cause congenital abnormalities.

37
Q

What is the mechanism of action of Mycophenolate?

A

Selective blockage of inosine monophosphate dehydrogenase, which produces a downregualtion of the pathway of purine synthesis of T and B cells.

38
Q

What are the side effects of Mycophenolate?

A

Gastro irritation
Risk of haematological malignancies
Leukopenia
Anaemia
Vomiting

39
Q

What are the unwanted side effects of prednisolone?

A

Weight gain
Electrolyte imbalance
Hypertension
GI discomfort
Osteoporosis
Cataract
Anxiety

40
Q

What biological therapies may be used in the treatment of Pemphigus and Pemphigoid?

A

Rituximab
Ofatumumab

41
Q

What is a biological medication?

A

Recombinant proteins interfere in an immunological process- i.e. monoclonal antibodies which target specific antigens.

42
Q

What is the first line treatment for Pemphigoid and Pemphigus?

A

Pemphigoid- prednisolone
Pemphigus- Rituximab

43
Q

What is the mechanism of action of Rituximab?

A

Monoclonal antibody for CD20-pisitive cells.

Causes B cell depletion by direct apoptosis, complement -dependent cytotoxicity and antibody phagocytosis.

44
Q

If someone is on Rituximab, what are they at increased risk of?

A

Opportunistic infections, Hep B, Hep C or TB.

45
Q

If a patient doesn’t respond well to Rituximab, what other biologic might they use?

A

Ofatumumab.

46
Q

In a general practice setting, what is the role of the GDP with regards to Pemphigus/Pemphigoid?

A

Benzydamine mouthwash- 0.15%, 300ml, gurgle 10 mln every 1-1.5 hours.

Betamethosone- 500 microgram tablets, dissolve in 10 mls of water, rinse 4 times a day

Beclomethasone- 50 micrograms, 200 dose unit, 1-2 puffs on the site twice daily.

Avoid spicy/hot foods, advise SLS free toothpaste

Refer patient Oral medicine and ophthalmologist if they have eye involvement.

Clinical photographs and measurements.

47
Q

What is Erythema Multiforme?

A

Spectrum disorder of immunogenic related skin and mucosa ulceration with variable orofacial involvement.

48
Q

Describe the pathogenesis of Erythema Multiforme?

A

Cutaneous and mucosal Type 3 or 4 hypersensitivity reaction depending on the causative factor.

Antibody attacks a circulating antigen- forms an antigen-antibody complex.
Complex is unable to pass through the capillaries, when it reaches the tissues, it becomes wedged in the blood vessels, resulting in peri-vascular inflammatory response.

When this is significant, the patient will get blistering or ulceration of the tissues.

The area of presentation depends on the antibody/antigen complex and the trigger for this.

49
Q

What type of patient is Erythema multiform most common in?

A

Young males- recurrent within a short period.

50
Q

What are the potential triggers of EM?

A

Drugs- antibiotics, carbamazepine, phenytoin, NSAIDs, statins, TNF-alpha inhibitors.
Herpes Simplex
Mycoplasma

51
Q

What are the signs and symptoms of someone with Erythema Multiforme?

A

Intra-orally- Blisters, which when broken down show shallow erosions.
White overlying pseudomembrane
Usually affects the lips (Haemorrhagic blistering of the lips) and anterior parts of the mouth.
Lesson on non-keratinised mucosa.

Painful, limit oral intake, difficulty swallowing.

Cutaneous features-
- Develops peripherally, then centrally.
- Symmetrical target lesions.
- Target lesions are round, erythematous papules with three concentric rings of colour variation.
- Central epidermal necrosis
- tighter oedematous area
- Peripheral erythematous margin.

Ulceration for 2-3 weeks.

52
Q

What group of patients does EM affect the most?

A

Young males- 20-40 years old.

53
Q

What group of patients are genetically predisposed to EM?

A

HLA-B12 antigen positive patients are 3 times more likely to develop EM.

54
Q

What medical conditions are associated with EM?

A

Herpes Simplex Virus

Influenza virus
Systemic Lupus Erythematosis
Epstein Barr virus
HIV
Hep C
Malignancies
Graft Vs Host disease
Candida infection

55
Q

In relation to EM, what is the difference between a Type 3 and Type 4 hypersensitivity reaction?

A

Type 3
- infection associated erythema multiforme
- Antibody mediated reaction, mediated by formation and accumulation of antigen-antibody aggregates, causing inflammation and tissue damage.

Type 4
- Drug hypersensitivity Erythema Multiforme
- Cell-mediated reaction in response to allergen, interaction of CD4+ helper T cells with antigens which produces an inflammatory response.

56
Q

What treatment is required for EM?

A

For oral lesions- systemic prednisolone (40-60mg/day), systemic acyclovir (if caused by HSV-1).

Encourage fluid intake.

Encourage analgesia.
- Benzydamine mouthwash- 0.15%.
- systemic analgesia.

Oral steroids.

Avoid any medication which is initiating the EM.

Refer to Oral medicine.

If recurrent problems- consider prophylactic acyclovir daily.

57
Q

What other drugs may be used for EM?

A

Mycophenolate
Azothioprine
Dapsone

58
Q

What are the histological features of EM?

A

Oral mucosa-
- Inter and intra cellular oedema of the overlying epithelium.
- Focal microvascular formation
- Keratin mucopolysacharide dystrophy
- Infiltration of mononuclear and polymorphonuclear cells into all epithelial layers.

Skin-
- Acanthosis
- Elongation of rate pegs
Satellite cell necrosis
- Vasodilation of blood vessels
- Diffuse infiltrate of mixed mononuclear cells of the upper portion of the lamina propria
Intraepithelial oedema and spongiosis

59
Q

What is Angina Bullosa Haemorrhagica?

A

Blood blisters in the mouth

60
Q

Where are ABH blisters commonly found?

A

Buccal mucosa and soft palate

61
Q

What are the signs and symptoms of ABH?

A

Blood filled blisters commonly found on the buccal mucosa and soft palate.

Rapid onset- appear within a few minutes, last about 1 hour then burst.

Relatively painless

Possibly initiated by minor trauma when eating

Heal with no scarring within a few days.

62
Q

What is the management for ABH?

A

No treatment available at present

Reassure patient that disease is benign

Explain triggers and course of disease- eating, steroid inhalers.

63
Q

What systemic diseases have oral effects?

A

Systemic Lupus Erythematosis
Systemic Sclerosis
Sjogren’s
Erythema Mulitforme
Pemphigus
Pemphigoid

64
Q

State local immunological oral diseases.

A

Lichen Planus
OFG
Aphthous ulcers

Oral Medicine (15 decks)

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