Vesiculobullous Conditions Flashcards
What is a Vesicle?
a small fluid filled blister <5mm
What is a Bulous?
a large fluid filled blister > 5mm
What is the epithelial classification of blistering?
- Intra-epithelial = fragile blisters
- Sub-epithelial = flimsy but long lasting blister
What 5 autoimmune blistering conditions?
1) Pemphigus Vulgaris (PV)
2) Mucous Membrane Pemphigoid (MMP)
3) Bullous Pemphigoid
4) Dermatitis Herpetiformis
5) Linear IgA disease
State 2 idiopathic blistering conditions?
Erythema Multiforme
Angina Bullosa Haemorrhagica (ABH)
State a collagen defect blistering condition?
Epidermolysis Bullosa
State 4 infectious (viral) blistering conditions?
- HSV
- HZV
- Hand,foot+ mouth disease (Coxsackie)
- Herpangia
What two conditions/types of blistering result in INTRA-EPITHELIAL BLISTERING?
- Pemphigus Vulgaris
- Viral infections
What investigations are used to diagnose blistering conditions?
- Biopsy - tissue sample (Direct immunofluorescence + histopathology)
- Blood Test (Indirect immunofluorescence + blood serum)
What type of investigation is direct immunofluorescence and give details about it?
= Biopsy
Method: Incisional biopsy (of lesions + normal skin)
Demonstrates bound immune complexes in mucosa or skin
What would a blood serum sample for blistering conditions show?
Conc. of haemotinics/ antibodies
What type of investigation is indirect immunofluorescence and give details about it?
= blood test (blood looked at under light)
Detects autoantibodies in serum
What is Pemphigus Vulgarus?
= autoimmune disease where IgG autoantibodies destroy desmosomes causing INTRA-EPITHELIAL blistering
(desmosomes are responsible for cell-cell adhesion i.e. it is the glue that holds cells together, igG autoantibody attacks desmosomes resulting in the break down of cell adhesion - thus intraepithelial blistering
we get blisters affecting oral mucosa WEEKS before anywhere else in the body; IO signs 1st)
Who does Pemphigus Vulgaris affect?
40-60yrs old
M=F
What is the aetiology (cause) of Pemphigus Vulgaris?
- genetic predisposition
- diet (e.g. garlic + red wine)
- Medication:
1. Sulphydryl drugs i.e. penicillamine
2. Non-thiol drugs w/ an active amide group i.e. diclofenac
What is the clinical presentation of Pemphigus Vulgarus?
DONE
Where?
- keratinised surfaces esp. on junction of hard + soft palate
- hard palate
- dorsum of tongue
- gingiva (+ buccal mucosa)
What?
- Oral bullae
- Nikolsky’s sign (oral blisters rapidly break down to form slow non-healing erosions/ulcer + white patches)
- Desquamative Gingivitis(in 60%)
- Erosive Lips
What investigations are used for diagnosis of Pemphigus Vulgarus?
- Incisional biopsy of fresh blister+ normal tissue for histology which shows “intra-epithelial clefting + acantholysis”
- Direct immunofluorescence (biopsy)
- Serum blood test for IgG autoantibody
- Indirect immunofluorescence (blood test)
What is the tx for Pemphigus Vulgaris?
1) Topical Corticosteroids (betamethasone m/w)
2) Systemic Corticosteroids (prednisolone)
3) Steroid sparing agents (azathioprine + cyclosporine)
4) Immunotherapy:
- IV monoclonal antibodies
- IV immunoglobulins
- Plasmapheresis
What is Mucous Membrane Pemphigoid? (MMP)
SUB-EPITHELIAL chronic blistering autoimmune disease
where the body attacks the basement membrane zone + epithelium lifts off underlying connective tissue (CT).
What is the aetiology of Mucous Membrane Pemphigoid? (MMP)
unknown aetiology
Who does Mucous Membrane Pemphigoid affect?
F> M
~ elderly 60yr old
What is the oral presentation of Mucous Membrane Pemphigoid?
Site:
any oral site
lip lesions = rare
Symptoms:
Large blood filled bullae that ruptures into chronic painful (pseudomembranous) erosions + ulcerated lesions
Desquamative gingivitis (blisters involving attached gingiva)
Nikolsky’s sign
Chronic Soreness
Dysphagia
KEY: may resemble LICHEN PLANUS
What is systemic presentation of Mucous Membrane Pemphigoid?
Occular involvement
- Visual impairment + blindness
- Chronic conjunctivitis
- one eye then both eyes in 2 years
- scarring then fibrosis
- vesicles/ ulcerations if very progressive
- skin (scalp + face + neck)
- Irreversible alopecia
- nose
- larynx + oesophagus
-genitals
What investigations are conducted for the diagnosis of Mucous Membrane Pemphagoid?
- Biopsy (histology -full thickness of epithelium lifts off CT
- Direct immunofluorescence - deposits of IgG/ C3
- Occular examination
What is the tx for Mucous Membrane Pemphagoid?
- Dapsone
- Topical corticosteroids (betamethasone m/w)
- Systemic corticosteroids (prednisolone if ocular involvement)
- anti-inflammatory antibiotics (doxycycline)
- immunosuppressant (non-steroidal i.e. azathioprine)
What is Bullous Pemphigoid?
= sub epithelial autoimmune blistering condition, erythema blisters that develop on skin (rarely mouth)
- initially itchy then erosions (scaring not prominent)
What is the management for Bullous pemphigoid?
Same investigations + tx as Mucous Membrane Pemphigoid
What is Dermatitis Herpetiformis?
= autoimmune sub-epithelial blistering disease assoc. w/ celiac disease
Who does Dermatitis Herpetiformis affect?
- younger age group
- (Irish people)
What is the cutaneous presentation of Dermatitis Herpetiformis?
chronic pruritic papulovesicular rash on the buttocks, elbows & knees, appears as small blisters on itchy base
What is the oral presentation of Dermatitis Herpetiformis?
transient superficial blisters (+ tender non-specific ulcers)
What is the management for Dermatitis Herpetiformis? a) investigations?
1.Histopathology (biopsy) → localised splitting at the basement
membrane zone (BMZ)
- Direct immunofluorescence (biopsy) → granular IgA immunofluorescent deposits involving the BMZ of the dermal papillae
- Blood serum test → positive for tissue transglutaminase IgA
What is the management for Dermatitis Herpetiformis? b) tx?
- Dietary restriction w/ gluten free diet
- Dapsone (gel or tablet)
What is Linear IgA Disease?
chronic autoimmune sub epithelial blistering mucocutaneous disease
- ORAL LESIONS present majority
- Ocular lesions infrequent
(not assoc/ w coeliac disease)
What is the management of Linear IgA disease?
Investigation:
- Direct immunofluorescence (biopsy) → linear deposits of IgA at CT
junction w/ separation at BMZ
Tx: dapsone / corticosteroids
What is Erythema Multiforme?
immune- mediated type III mucocutaneous blistering condition, triggered by:
- Infections (e.g. recurrent HSV, HZV)
- Drugs (antiretrovirals, antibxs, antifungals, NSAIDS, barbiturates)
- Systemic e.g. pregnancy, malignancy, SLE
- Idiopathic
Who does Erythema Multiforme affect?
<30 yr old & M>F
What is the cutaneous presentation of Erythema Multiforme?
central blister surrounded by oedematous ring with erythematous border = target lesion
Where? Skin>palms>soles
What is the oral presentation of Erythema Multiforme?
bullae that rapidly break into irregular ulcers, bleed, form crusts (gingiva rarely affected)
Lips + palate most affected
What is the tx for Erythema Multiforme?
Tx:
- Usually self-resolving
- Acyclovir 5% topical cream AT BEGINNING STAGE
- Penciclovir 1% cream, low compliance due to re-application every 2 hrs
- Coffee grounds ?
- Consider systemic acyclovir 400mg x2 day if the pt has recurrent HSV
What is Angina Bullosa Haemorrhagica? (ABH)
= Chronic idiopathic sub-epithelial condition causing blood blisters
Who does Angina Bullosa Haemorrhagica affect?
Elderly
Steroid spray users (asthmatics)
Diabetics
Genetic predisposition
What is the oral presentation of Angina Bullosa Haemorrhagica?
- sudden appearance of blood filled blisters (often associated w/ consumption of dry/rough food, pt feels like they’re choking)
- solitary (alone)
- 2-3cm diameter (quite large)
-bursts spontaneously ~24hrs, heals in 7-10days w/o scarring - recurs every few years
SITE: junction of hard + soft palate
What is the management of ABH?
Investigations:
- FBC/Coagulation screen
Tx:
- Benzydamine & chlorhexidine m/w
- Incise large intact blood blister (to prevent pt from choking feeling &
respiratory embarrassment)
What is Epidermolysis Bullosa?
= sub epithelial collagen defect blistering condition
who?
Any age
F>M
What are the 3 forms of Epidermolysis Bullosa?
- EB simplex – least severe
- EB dystrophica [genetic] – fragile skin & mucosa
- EB lethalis – lethal/deadly
What is the clinical presentation of Epidermolysis Bullosa?
Any trauma to skin or mucosa resulting in separation of underlying CT, then scaring and deformity
There OH + eating is problematic
What is the management of Epidermolysis Bullosa?
Investigations:
- Direct immunofluorescence → linear IgG deposits & collagen type
7 anchoring fibril target antigen
Tx:
- Oral corticosteroids
- Immunosuppressives +/- dapsone
