Mucocutaneous Disease Flashcards

1
Q

What is a macule?

A

Circumscribed flat lesion

Not elevated and not palpable

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2
Q

What is papule?

A

Circumscribed raised lesion

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3
Q

What is blister?

A

Fluid filled sac in epithelium or bone

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4
Q

What is vesicle?

A

Small blister less than 5mm in size

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5
Q

What is bulla?

A

Large blister >5mm in size

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6
Q

What is erosion?

A

Marked thinning or partial loss of epithelium - thin covering remains

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7
Q

What is ulcer?

A

Localised loss of entire thickness of epithelium exposing underlying CT

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8
Q

Examples of autoimmune bullous disease?

A

Pemphigus
Pemphigoid
Dermatitis herpetiformis

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9
Q

What type of hypersensitivity are autoimmune bullous disease and why?

A

Type II as antibody mediated and organ specific

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10
Q

What is pemphigus?

A

Organ specific autoimmune disease targeting skin and oral mucosa

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11
Q

Who more likely to see pemphigus in?

A

40-60year olds

Male: = female

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12
Q

Are lesions always seen in mouth in pemphigus cases?

A

Involved most cases

In 50% of cases only site affected

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13
Q

Where in mouth expect to see bulla of pemphigus?

A

Palate, buccal mucosa and gingival - often short lived

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14
Q

How would expect bulla appear in pemphigus?

A

Large shallow non-healing ulcer

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15
Q

How expect gingiva to look in pemphigus?

A

Full thickness inflammation - gingivitis

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16
Q

What clinical test is used in pemphigus?

A

Nikolsky’s sign

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17
Q

What is Nikolsky’s sign?

A

Use blunt end of probe (not on blister) to see if underlying bullies problem

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18
Q

What will positive Nikolsky’s sign show?

A

Skin will shear off

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19
Q

What is pathogenesis of pemphigus?

A

Have circulating antibodies against binding proteins desmosomes
Autoantibody binds to desmoglein 3 causing acantholysis and formation if intra-epithelial bulla

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20
Q

What bulla do see in pemphigus?

A

Intra-epithelial

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21
Q

What see histology of pemphigus regarding basement membrane?

A

Basement membrane is attached to connective tissue

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22
Q

Where see bulla in histology of pemphigus?

A

Intra-epithelial - see cleft/split supra-basal therefore within epithelial cells

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23
Q

What cells might expect to see in split on pemphigus histology?

A

Tzank cells

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24
Q

What investigations would be required to test for pemphigus?

A

Biopsy for histology
Direct immunofluorsence staining
Blood sample

25
Q

How take biopsy to confirm pemphigus?

A

Take para-lesionsal or normal tissue - don’t biopsy that blister
Send as fresh sample

26
Q

What does direct immunofluorsence staining do?

A

detect which antibodies present

27
Q

What would be looking for in blood sample when checking for pemphigus?

A

Circulating demoglein levels

28
Q

What expect to see from immuoflurosence from pemphigus sample?

A

Positive would show staining in epithelial cells revolting fish net pattern

29
Q

What is direct immunoflurosence?

A

Use biopsy sample which will have auto-antibodies (if +ve) then use flurosent label to identify desmoglein abs

30
Q

What is indirect immunoflurosence?

A

Use slide w/ control tissue then use pt serum sample- serum will bind to normal tissue - add labels IgG

31
Q

What are 3 subtypes of pemphigus?

A
  1. Pemphigus vulgaris
  2. Pemphigus foliaceous - superficial lesions
  3. Paraneoplastic pempgius
32
Q

Most common type of pemphigus?

A

Pemphigus vulgaris

33
Q

Management of pemphigus?

A

Immunosuppression - help manage sympotoms - prednisolone +- azathioprine
EXCLUDE CANCER

34
Q

Issue w/ pemphigus and intra-epithelial bulla?

A

Basement membrane remained attached CT therefore no stimulus to heal
Epithelial permeability barrier compromised allowing increased risk of infection and loss of tissue fluid

35
Q

See in intra-epithelial bulla?

A

Fragile bulla where basement membrane remains attached to CT

36
Q

When see sub-epithelial bulla?

A

Pemphigoid

37
Q

What is a sub-epithelial bulla?

A

Bulla between epithelium, and connective tissue

38
Q

What happens if sub-epithelial bulla ruptures?

A

Exposes underlying connective tissue which heals by secondary intention

39
Q

What disease see sub-epithelial bulla?

A

Pemphigoid
Mucous membrane pemphigus
Dermatitis herpetiformis

40
Q

Pathogenesis of pemphigoid?

A

Circulating autoantibodies against hemidesmosomes

41
Q

What see is histology of pemphigoid?

A

Epithelium is separate from connective tissue at level of basement membrane - no basement membrane attached CT

42
Q

What is mucous membrane pemphigoid?

A

Often chronic disease of elderly

43
Q

What features see mucous membrane pemphigoid?

A

Desquamative gingivitis in 90% case

Eye lesions that can cause scarring

44
Q

Do have skin lesions in MMP?

A

Very rare

45
Q

What types of lesion seen in MMP?

A

Well marginated ulcers

46
Q

Treatment of MMP?

A

Steroids - topical or systemic
OHI - plaque reduction
Immunosuppressive agents
Opthamological assessment

47
Q

What expect to see in bullous pemphigoid?

A

Skin usually involved w/ large and shallow ulcers and erosions

48
Q

Investigations for pemphigoid?

A

Biopsy
Routine histology
Direct immunoflurosence

49
Q

What expect to see in immunoflurosence for pemphigoid?

A

Don’t see fishnet, linear pattern along basement membrane

50
Q

What type of bullous disease can be associated w/ coeliac?

A

Dermatitis herptiformis

51
Q

Who does dermatitis herptiformis affect?

A

Younger patients

52
Q

What is herpetiformis dermatitis?

A

Bullous disorder w/ skin and oral lesions

53
Q

What see histology of herpetiformis dermatitis?

A

Small regions sub-epithelial separation at level of basement membrane
In blister see neutrophils and eosinophils

54
Q

What is epidermolysis bulls congenita?

A

Inherited bullous disorder caused by genetic defect in key proteins associated w/ epithelial integrity

55
Q

What is erythema multiforme?

A

Mucocutaneous blistering disorder w/ acute onset and short duration

56
Q

Oral clinical features of erythema multiforme?

A

Haemorrhagic crusting of lips

Irregular mucosal ulceration

57
Q

What other clinical features are seen in erythema multiforme?

A

Occlusal lesions

‘target’ characteristic lesions

58
Q

Management of erythema multiforme?

A

Remove/ avoid triggers
Short course steroids
CHX mouthwash
Analgesia advice inc soft diet