Blistering Disorders Flashcards

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1
Q

Where histologically is the origin/aetiology of Mucous Membrane Pemphigoid?

A

Sub-Epithelial

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2
Q

What type of disease is erythema multiforme?

What type of reaction is it

A

Immune-mediated Type III Mucocutaneous blistering reaction

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3
Q

Define Bulla

A

Large fluid-filled blister
(over 5mm diameter)

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4
Q

Ideally when investigating a blistering disorder what would you want to biopsy?

A

Small fresh blister

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5
Q

Describe the appearance of a cutaneous lesion for erythema multiforme

A

Macules and papules which have a pale area surrounded by oedema and bands of erythema

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6
Q

What is this?

A

Erythema Multiforme

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7
Q

What is this?

A

Desquamative gingivitis

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8
Q

How does dermatitis herpetiformis present?

A

Chronic pruritic (itchy) papulo-vesicular rash

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9
Q

What is this?

A

Pemphigus vulgaris

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10
Q

What is the treatment of dermatitis herpetiformis?

A

Gluten-free diet & Dapsone

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11
Q

What is an indirect immunofluorescence investigation?

Go through the use and method

A

Detects autoantibodies in serum

Method:
1. Serum sample taken & sections of NORMAL mucosa/skin taken & put on slide
2. Primary incubation
3. Secondary incubation with FITC (fluorescent marker) for anti-human IgG, IgA & C3

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12
Q

Where orally does angina bullosa haemorrhagica present?

A

Junction between hard & soft palate

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13
Q

What disease is Dermatitis Herpetiformis associated with?

A

Associated with Gluten-sensitive Enteropathy (Coeliac Disease)

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14
Q

What is this?

A

Desquamative gingivitis

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15
Q

At what age does mucous membrane pemphigoid present?

A

Middle age

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16
Q

What is Nikolsky’s sign?

A

Oral blisters rapidly break down to form erosions

Associated with pemphigus vulgaris

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17
Q

What investigations would you take when suspected Mucous Membrane Pemphigoid?

A

Histopatholgy
Direct immunofluorescence

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18
Q

What investigations would you take when suspected pemphigus vulagris?

A

Routine histology
Direct and Indirect Immunofluorescence

19
Q

What is this?

A

Mucous membrane pemphigoid

20
Q

What are common sites for dermatitis herpetiformis presentation?

A

Buttocks, elbows and knees

21
Q

What is this?

A

Erythema multiforme

22
Q

What type of drugs may cause pemphigus vulgaris?

A

Sulphydryl containing drugs
Non-thiol drugs

23
Q

What is the clinical presentation of Angina Bullosa Haemorrhagica?

A

Sudden appearance of solitary large blood-filled blisters (2-3cm diameter)

Intraoral blood blisters

24
Q

What age is common for erythema multiforme presentation?

A

Under 20

25
Q

What are the clinical features of pemphigus vulgaris? (3)

A

Oral bullae
Nikolsky’s sign (Oral blisters rapidly break down to form erosions)
Desquamative gingivitis (in 60%)

26
Q

Where histologically is the origin/aetiology of Pemphigus Vulgaris?

A

Intra-Epithelial

27
Q

What is the clinical presentation of mucous membrane pemphigoid? (3)

A

Large blood filled blisters that rupture and leave erosions
Desquamative gingivitis (blisters involving attached gingivae)
Nikolsky’s sign

28
Q

What are some treatments for mucous membrane pemphigoid?

A

Topical corticosteroids
Systemic corticosteroids
Dapsone (anti-bacterial antibiotic)

29
Q

What is the treatment for pemphigus vulgaris?

A

Systemic glucocorticosteroids & steroid sparring agents

30
Q

What can be occular complications of mucous membrane pemphigoid?

A

Chronic conjunctivitis which leads to scarring (ie cicatricial conjunctivitis) this results in entropion (inturning of lower eyelid) can progress to blindness

31
Q

What is the peak incidence of pemphigus vulgaris?

A

40-60 years old

32
Q

What is the management of angina bullosa haemorrhagica?

A

FBC & Coagulation screen
Large intact blood-blister incisal (to avoid respiratory obstruction)
Symptomatic Tx (e.g. Difflam benzydamine or CHX mouthwash)

33
Q

Define vesicle

A

Small fluid-filled blister
(under 5mm diameter)

34
Q

What occurs (at cellular level) for a patient with Mucous membrane pemphigoid?

A

Circulating & tissue-bound IgG autoantibodies harm basement membrane zone

35
Q

What medical condition is linked with angina bullosa haemorrhagica?

A

Diabetes

36
Q

What is the most common cause of erythema multiforme?

A

HSV

37
Q

Where histologically is the origin/aetiology of mucous membrane Pemphigoid?

A

Sub-Epithelial

38
Q

What is a direct immunofluorescence investigation?

Go through what it shows and the method

A

Demonstrates bound immune complexes in mucosa or skin

Method:
1. Incisional biopsy (of lesion AND normal skin)
2. Biopsy sectioned on slide
3. Incubation with FITC (fluorescent marker) for anti-human IgG, IgA, IgM & complement

39
Q

What are the oral features of Epidermolysis Bullosa?

A

Fragility of skin / oral mucosa so minor trauma causes skin or oral mucosa to separate from underlying connective tissue

The resulting scarring may lead to severe deformity

40
Q

What is this?

A

Pemphigus vulgaris

41
Q

What is this?

A

Dermatitis herpetiformis

42
Q

What is a common oral feature of erythema multiforme?

A

Lip crusting

Bullae break rapidly into irregular ulcers, bleed, form crusts

43
Q

Where are common sites for cutaneous lesions for erythema multiforme?

A

Often affects extremities (palms & soles)

44
Q

What occurs (at cellular level) for a patient with pemphigus vulgaris?

A

Circulating & tissue-bound IgG autoantibodies harm adhesion proteins of desmosomes which distrupts cell-to-cell adhesion

Leading to intraepithelial blisters affecting the skin and mucosa