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Blistering Disorders Flashcards

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

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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?

25
What are the clinical features of pemphigus vulgaris? (3)
Oral bullae Nikolsky’s sign (Oral blisters rapidly break down to form erosions) Desquamative gingivitis (in 60%)
26
Where histologically is the origin/aetiology of Pemphigus Vulgaris?
Intra-Epithelial
27
What is the clinical presentation of mucous membrane pemphigoid? (3)
Large **blood filled** blisters that rupture and leave erosions Desquamative gingivitis (blisters involving attached gingivae) Nikolsky’s sign
28
What are some treatments for mucous membrane pemphigoid?
Topical corticosteroids Systemic corticosteroids Dapsone (anti-bacterial antibiotic)
29
What is the treatment for pemphigus vulgaris?
Systemic glucocorticosteroids & steroid sparring agents
30
What can be occular complications of mucous membrane pemphigoid?
Chronic conjunctivitis which leads to scarring (ie cicatricial conjunctivitis) this results in entropion (inturning of lower eyelid) can progress to blindness
31
What is the peak incidence of pemphigus vulgaris?
40-60 years old
32
What is the management of angina bullosa haemorrhagica?
FBC & Coagulation screen Large intact blood-blister incisal (to avoid respiratory obstruction) Symptomatic Tx (e.g. Difflam benzydamine or CHX mouthwash)
33
Define vesicle
Small fluid-filled blister (under 5mm diameter)
34
What occurs (at cellular level) for a patient with Mucous membrane pemphigoid?
Circulating & tissue-bound IgG autoantibodies harm basement membrane zone
35
What medical condition is linked with angina bullosa haemorrhagica?
Diabetes
36
What is the most common cause of erythema multiforme?
HSV
37
Where histologically is the origin/aetiology of mucous membrane Pemphigoid?
Sub-Epithelial
38
What is a direct immunofluorescence investigation? | Go through what it shows and the method
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
What are the oral features of Epidermolysis Bullosa?
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
What is this?
Pemphigus vulgaris
41
What is this?
Dermatitis herpetiformis
42
What is a common oral feature of erythema multiforme?
Lip crusting | Bullae break rapidly into irregular ulcers, bleed, form crusts
43
Where are common sites for cutaneous lesions for erythema multiforme?
Often affects extremities (palms & soles)
44
What occurs (at cellular level) for a patient with pemphigus vulgaris?
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
45
What are the causes of EM?
- Infection- HSV (70%), hepatitis viruses, mycoplasma, bacterial, fungal, parasites - Drugs- NSAIAs, antifungals, barbiturates - Systemic- SLE, malignancy, pregnancy - Idiopathic- 50%
46
What is the aetiology of angina bullosa haemorrhagica (ABH)
- Genetic predisposition loose adhesion between epithelium and corium of the mucosa or a weak anchorage of mucosal vessels may result in subepithelial haemorrhage - Inhaled corticosteroids mucosal atrophy causes decreased submucosal elastic fibres , especially in elderly population >5 years. Long term use may induce capillary breakdown - Diabetes mellitus may also cause vascular fragility
47
What is the tx for epidermolysis bullosa acquisita (EBA)?
Treatment: difficult oral corticosteroids + immunosuppressives dapsone

Oral Med (17 decks)

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