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BDS4 BAMS Oral Medicine > 5. Vesiculobullous & Immune Disease > Flashcards

5. Vesiculobullous & Immune Disease Flashcards

1
Q

Define vesicle.

A

<5-10mm in size.

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2
Q

Define bullae.

A

> 10mm in size.

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3
Q

What type of hypersensitivity reaction is erythema multiforme ?

A

Type 3

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4
Q

SPECIAL INVESTIGATIONS

What is immunofluorescence ?

A
  • Permits visualisation of components in tissue/cell type.
  • Identifies presence of antibodies.
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5
Q

SPECIAL INVESTIGATIONS

When is direct immunofluorescence preferred ?

A

In diagnosis of disease.

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6
Q

SPECIAL INVESTIGATIONS

When is indirect immunofluorescence preferred ?

A

Monitoring disease activity and determining treatment need.

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7
Q

SPECIAL INVESTIGATIONS

What is direct immunofluorescence ?

A

Where antibody is bound to tissue.

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8
Q

SPECIAL INVESTIGATIONS

What is indirect immunofluorescence ?

A

Circulating antibody not yet bound to tissue (detected by immunofluorescence from plasma sample).

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9
Q

ERYTHEMA MULTIFORME

Does it affect males or females more ?

A

Males (young males late teens/early 20s).

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10
Q

ERYTHEMA MULTIFORME

What are the aetiological factors ?

A

Reactivation of herpes simplex virus.

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11
Q

ERYTHEMA MULTIFORME

Symptoms/signs ?

A

Target like skin lesions.
Crusting of the lips.
Ulceration of the mouth affecting keratinised and non-keratinised tissue.
Unable to eat/drink - due to pain.
Looks similar to primary herpetic stomatitis.

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12
Q

ERYTHEMA MULTIFORME

Explain how the type 3 hypersensitivity reaction occurs ?

A
  1. Antigen (present in body before) targeted by antibodies causing prompt immune response.
  2. Antigen-antibody complex formed - large and cannot pass through capillaries.
  3. Becomes lodged in capillaries when passing through tissues.
  4. Activates BVs causing perivascular inflammatory response.
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13
Q

ERYTHEMA MULTIFORME

What is the name for erythema multiforme in its extreme form (multisystem involvement) ?

A

Stevens Johnson syndrome.

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14
Q

ERYTHEMA MULTIFORME

You work in secondary care setting and a patient presents with EM - what treatment can you offer ?

A

High dose prednisolone - suppress immune reaction - up to 60mg/day.
Acyclovir prophylaxis - prevent reactivation of virus - 400mg 2x daily.
Encourage hydration - maybe admission for IV ?
Encourage analgesia.

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15
Q

ERYTHEMA MULTIFORME

What is erythema multiforme ?

A

Spectrum disorder of immunogenic related skin and mucosal ulceration (with variable orofacial involvement).

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16
Q

ANGINA BULLOUSA HAEMORRHAGICA

What is angina bullosa haemorrhagica (ABH) ?

A

Formation of tight, blood filled blister on oral mucosa.

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17
Q

ANGINA BULLOUSA HAEMORRHAGICA

Where in the mouth does ABH most commonly affect ?

A

Buccal mucosa, soft palate.

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18
Q

What is the most common oral blistering condition ?

A

ABH

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19
Q

ANGINA BULLOUSA HAEMORRHAGICA

Signs/symptoms ?

A

Blood filled blisters with rapid onset (in minutes) and last around an hour and then burst.
Painless.
Heal with no scarring.

20
Q

ABH - aetiology ?

A

No obvious etiological factor.
Minor trauma ?
Inhalers ?

21
Q

ANGINA BULLOUSA HAEMORRHAGICA

What is found on biopsy ?

A

Non-specific ulceration.
DIF, IIF negative.
No platelet/coagulation defect.

22
Q

ANGINA BULLOUSA HAEMORRHAGICA

What are the only treatment options for angina bullosa haemorrhagica ?

A

Symptomatic relief - chlorhexidine or diflam spray.
Reassure patient that it is benign.

23
Q

PEMPHIGOID/PEMPHIGUS

What is pemphigoid ?

A

Immune mediated antibody-directed disease - “sub-epithelial antibody attack” - causing antibody production against desmosomes (which join basement membrane and CT).

24
Q

PEMPHIGOID/PEMPHIGUS

Explain how pemphigoid results in blistering ?

A

Antibodies cause separation of epithelium at basement membrane from CT.
Inflammatory exudate fills space between epidermis and CT.
Blisters appear.

25
Q

PEMPHIGOID/PEMPHIGUS

Define the type of blistering found in pemphigoid.

A

Thick walled blisters (full epidermis).
Clear or red.
Persist for long time.

26
Q

PEMPHIGOID/PEMPHIGUS

What are three different types/presentations of pemphigoid ?

A

Bullous pemphigoid (skin).
Mucous membrane pemphigoid (eyes, genitals, mouth).
Cicatritial pemphigoid (leaves scarring).

27
Q

PEMPHIGOID/PEMPHIGUS

Pemphigoid - what might be seen histopathologically ?

A
  • Sub-epithelial split between epithelium and CT junction.
  • Hemi-desmosomes involved at basement membrane.
  • Superficial perivascular inflammatory infiltrate.
  • Inflammatory exudate containing fibrin, erythrocytes and inflammatory cells within cleft.
28
Q

PEMPHIGOID/PEMPHIGUS

Pemphigoid - what is the most useful diagnostic test ?

A

Immunofluorescence.

29
Q

PEMPHIGOID/PEMPHIGUS

Pemphigoid - what might be seen on immunofluorescence ?

A

Linear staining along basement membrane.
C3 and IgG detected in standard pemphigoid (IgA occasionally - will have patchy appearance).

30
Q

PEMPHIGOID/PEMPHIGUS

In a secondary care setting, how should pemphigoid be managed ?

A

Topical steroids - tacrolimus.
Systemic steroids - prednisolone.
Immune modulating drugs - azathioprine, mycophenolate, dapsone.

31
Q

PEMPHIGOID/PEMPHIGUS

Define pemphigus.

A

Immune mediated antibody-directed disease - where antibody is produced against desmosomes which join epithelial cells together.
Causes epithelial cells to lose adhesion and intraepithelial bullae to form.

32
Q

PEMPHIGOID/PEMPHIGUS

How does pemphigus present ?

A

Thinning of epithelium progressing to loss of epithelium (rare to see intact bullae).
Therefore - mucosal erosion and mucosal surface loss.

33
Q

PEMPHIGOID/PEMPHIGUS

What is the difference between pemphigus vs. pemphigoid ?

A

Pemphigoid - intact bullae will be visible.
Pemphigus - erosive presentation.

34
Q

PEMPHIGOID/PEMPHIGUS

What is the main concern associated with both pemphigoid and pemphigus ?

A

Fluid loss and infection risk.

35
Q

PEMPHIGOID/PEMPHIGUS

What is the most common type of pemphigus ?

A

Pemphigus vulgaris.

36
Q

PEMPHIGOID/PEMPHIGUS

What age bracket does pemphigus affect most commonly ?

A

50+

37
Q

PEMPHIGOID/PEMPHIGUS

Does pemphigus affect males or females more commonly ?

A

Females.

38
Q

PEMPHIGOID/PEMPHIGUS

What particular genetic and racial group has a predisposition to developing pemphigus ?

A

Ashkenazi jews.

39
Q

PEMPHIGOID/PEMPHIGUS

What sites does pemphigus affect ?

A

Oral (usually first) then skin (both will eventually become involved).

40
Q

PEMPHIGOID/PEMPHIGUS

Pemphigus - around what time does skin involvement occur post-oral involvement ?

A

3 years

41
Q

PEMPHIGOID/PEMPHIGUS

Pemphigus - what will be seen histopathologically ?

A

Supra-basal split - occurring above basement membrane.
Tzank cells.

42
Q

PEMPHIGOID/PEMPHIGUS

Pemphigus - what will be seen on immunofluorescence ?

A

Basket-weave pattern.
C3 and IgG.

43
Q

PEMPHIGOID/PEMPHIGUS

What type of biopsy should be taken for pemphigus and pemphigoid ?

A

Perilesional biopsy.

44
Q

PEMPHIGOID/PEMPHIGUS

What are the signs/symptoms of pemphigus ?

A

Erosive lesions.
Appears like desquamative gingivitis.
Affects any part of oral mucosa.
Rarely see intact bullae.

45
Q

PEMPHIGOID/PEMPHIGUS

You are treating a patient with pemphigus vulgaris in secondary care setting - what treatment can you provide ?

A

High dose systemic steroids - prednisolone.
Immunosuppressants - azathioprine and mycophenolate.

BDS4 BAMS Oral Medicine (9 decks)

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