Blistering Disorders Flashcards
Where histologically is the origin/aetiology of Mucous Membrane Pemphigoid?
Sub-Epithelial
What type of disease is erythema multiforme?
What type of reaction is it
Immune-mediated Type III Mucocutaneous blistering reaction
Define Bulla
Large fluid-filled blister
(over 5mm diameter)
Ideally when investigating a blistering disorder what would you want to biopsy?
Small fresh blister
Describe the appearance of a cutaneous lesion for erythema multiforme
Macules and papules which have a pale area surrounded by oedema and bands of erythema
What is this?
Erythema Multiforme
What is this?
Desquamative gingivitis
How does dermatitis herpetiformis present?
Chronic pruritic (itchy) papulo-vesicular rash
What is this?
Pemphigus vulgaris
What is the treatment of dermatitis herpetiformis?
Gluten-free diet & Dapsone
What is an indirect immunofluorescence investigation?
Go through the use and method
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
Where orally does angina bullosa haemorrhagica present?
Junction between hard & soft palate
What disease is Dermatitis Herpetiformis associated with?
Associated with Gluten-sensitive Enteropathy (Coeliac Disease)
What is this?
Desquamative gingivitis
At what age does mucous membrane pemphigoid present?
Middle age
What is Nikolsky’s sign?
Oral blisters rapidly break down to form erosions
Associated with pemphigus vulgaris
What investigations would you take when suspected Mucous Membrane Pemphigoid?
Histopatholgy
Direct immunofluorescence
What investigations would you take when suspected pemphigus vulagris?
Routine histology
Direct and Indirect Immunofluorescence
What is this?
Mucous membrane pemphigoid
What are common sites for dermatitis herpetiformis presentation?
Buttocks, elbows and knees
What is this?
Erythema multiforme
What type of drugs may cause pemphigus vulgaris?
Sulphydryl containing drugs
Non-thiol drugs
What is the clinical presentation of Angina Bullosa Haemorrhagica?
Sudden appearance of solitary large blood-filled blisters (2-3cm diameter)
Intraoral blood blisters
What age is common for erythema multiforme presentation?
Under 20
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%)
Where histologically is the origin/aetiology of Pemphigus Vulgaris?
Intra-Epithelial
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
What are some treatments for mucous membrane pemphigoid?
Topical corticosteroids
Systemic corticosteroids
Dapsone (anti-bacterial antibiotic)
What is the treatment for pemphigus vulgaris?
Systemic glucocorticosteroids & steroid sparring agents
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
What is the peak incidence of pemphigus vulgaris?
40-60 years old
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)
Define vesicle
Small fluid-filled blister
(under 5mm diameter)
What occurs (at cellular level) for a patient with Mucous membrane pemphigoid?
Circulating & tissue-bound IgG autoantibodies harm basement membrane zone
What medical condition is linked with angina bullosa haemorrhagica?
Diabetes
What is the most common cause of erythema multiforme?
HSV
Where histologically is the origin/aetiology of mucous membrane Pemphigoid?
Sub-Epithelial
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
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
What is this?
Pemphigus vulgaris
What is this?
Dermatitis herpetiformis
What is a common oral feature of erythema multiforme?
Lip crusting
Bullae break rapidly into irregular ulcers, bleed, form crusts
Where are common sites for cutaneous lesions for erythema multiforme?
Often affects extremities (palms & soles)
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
