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
How take biopsy to confirm pemphigus?
Take para-lesionsal or normal tissue - don't biopsy that blister Send as fresh sample
26
What does direct immunofluorsence staining do?
detect which antibodies present
27
What would be looking for in blood sample when checking for pemphigus?
Circulating demoglein levels
28
What expect to see from immuoflurosence from pemphigus sample?
Positive would show staining in epithelial cells revolting fish net pattern
29
What is direct immunoflurosence?
Use biopsy sample which will have auto-antibodies (if +ve) then use flurosent label to identify desmoglein abs
30
What is indirect immunoflurosence?
Use slide w/ control tissue then use pt serum sample- serum will bind to normal tissue - add labels IgG
31
What are 3 subtypes of pemphigus?
1. Pemphigus vulgaris 2. Pemphigus foliaceous - superficial lesions 3. Paraneoplastic pempgius
32
Most common type of pemphigus?
Pemphigus vulgaris
33
Management of pemphigus?
Immunosuppression - help manage sympotoms - prednisolone +- azathioprine EXCLUDE CANCER
34
Issue w/ pemphigus and intra-epithelial bulla?
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
See in intra-epithelial bulla?
Fragile bulla where basement membrane remains attached to CT
36
When see sub-epithelial bulla?
Pemphigoid
37
What is a sub-epithelial bulla?
Bulla between epithelium, and connective tissue
38
What happens if sub-epithelial bulla ruptures?
Exposes underlying connective tissue which heals by secondary intention
39
What disease see sub-epithelial bulla?
Pemphigoid Mucous membrane pemphigus Dermatitis herpetiformis
40
Pathogenesis of pemphigoid?
Circulating autoantibodies against hemidesmosomes
41
What see is histology of pemphigoid?
Epithelium is separate from connective tissue at level of basement membrane - no basement membrane attached CT
42
What is mucous membrane pemphigoid?
Often chronic disease of elderly
43
What features see mucous membrane pemphigoid?
Desquamative gingivitis in 90% case | Eye lesions that can cause scarring
44
Do have skin lesions in MMP?
Very rare
45
What types of lesion seen in MMP?
Well marginated ulcers
46
Treatment of MMP?
Steroids - topical or systemic OHI - plaque reduction Immunosuppressive agents Opthamological assessment
47
What expect to see in bullous pemphigoid?
Skin usually involved w/ large and shallow ulcers and erosions
48
Investigations for pemphigoid?
Biopsy Routine histology Direct immunoflurosence
49
What expect to see in immunoflurosence for pemphigoid?
Don't see fishnet, linear pattern along basement membrane
50
What type of bullous disease can be associated w/ coeliac?
Dermatitis herptiformis
51
Who does dermatitis herptiformis affect?
Younger patients
52
What is herpetiformis dermatitis?
Bullous disorder w/ skin and oral lesions
53
What see histology of herpetiformis dermatitis?
Small regions sub-epithelial separation at level of basement membrane In blister see neutrophils and eosinophils
54
What is epidermolysis bulls congenita?
Inherited bullous disorder caused by genetic defect in key proteins associated w/ epithelial integrity
55
What is erythema multiforme?
Mucocutaneous blistering disorder w/ acute onset and short duration
56
Oral clinical features of erythema multiforme?
Haemorrhagic crusting of lips | Irregular mucosal ulceration
57
What other clinical features are seen in erythema multiforme?
Occlusal lesions | 'target' characteristic lesions
58
Management of erythema multiforme?
Remove/ avoid triggers Short course steroids CHX mouthwash Analgesia advice inc soft diet