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what is a vesicle?
- circumscribed skin lesion containing fluid
- <5mm
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what is a bulla?
- circumscribed skin lesion containing fluid
- >5mm
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what is the most common cause of blistering in skin?
extrinsic - eg trauma, burns, insect bites
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what are the inflammatory causes of blistering?
eczema - pompholyx
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what are the infectious causes of blistering?
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what are the autoimmune causes of blistering?
- bullous pemphigus
- bullous pemphigoid
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what are the main Ix done for blistering disease?
- skin biopsy: haematoxylin & eosin; immunofluorescence (direct)
- blood tests: immunofluorescence (indirect), porphyria screen
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what are the 4 main aspects of caring for blister patient?
- DEROOF: puncture and deflate blister
- potassium permanganate soaks
- regular emollients
- treat cause
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how do you treat pompholyx eczema?
high dose steroids
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what age group does bullous pemphigoid occur in?
older patients
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what is cause of bullous pemphigoid?
- autoimmune
- antibodies against
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how does bullous pemphigoid start?
itchy, urticarial plaques in limbs
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what happens later in bullous pemphigoid? describe what you see
develop TENSE intact blisters
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what is the distribution of rash in bullous pemphigoid?
generalised
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apart from skin where else may you see evidence of bullous pemphigoid?
oral mucosa - rare
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what is the course of bullous pemphigoid?
self limiting
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when can Rx for bullous pemphigoid be stopped?
around 2 years
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what is Rx of bullous pemphigoid?
deroof, soak in Kpermang, steroids
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what are the 3 complications of bullous pemphigoid?
- discomfort
- loss of fluid from ruptured bulla
- infection
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which is more aggressive bullous pemphigoid or pemphigus vulgarise?
pemphigus vulgaris
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what is the mortality rate of untreated pemphigus vulgarise?
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where does pemphigus vulgaris start?
oral mucosa - commonly affects mucous membranes (unlike pemphigoid)
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what is cause of pemphigus vulgaris? how common?
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how would you describe the vesicles in pemphigus vulgaris? what does it feel to patient?
- flaccid vesicles that form into erosions
- PAINFUL
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where is Rx of pemphigus vulgaris?
- aggressive immunosuppression
- prednisolone
- mycophenolate
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what are the 2 main complications of pemphigus vulgarise?
- overwhelming sepsis as massive immunosuppression
- drug side effects
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what age does pemphigus vulgaris affect?
middle aged
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which sign is positive in pemphigus vulgaris and what does this mean?
- Nikolsky's sign
- bullae are easily broken
- even rubbing apparently normal skin causes the superficial epidermis to slough off
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where are the autoantibodies attacking in pemphigus?
INTRAepidermal
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where are the autoantibodies attacking in pemphigoid?
SUBepidermal
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if a patient presents with diarrhoea, weight loss and a vesicular rash on extensors what is it?
- dermatitis herpetiformis
- associated with coeliac disease - gluten sensitive enteropathy
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what is the main symptom of dermatitis herpetiformis?
itchy
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what age and gender and race does dermatitis herpetiformis affect mostly?
- early adult life 20's
- m>f 2:1
- caucasians
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how would you describe the rash of dermatitis herpetiformis - inc morphology and distribution
- papules and vesicles
- excoriated
- symmetrical
- extensors - elbow, knee, buttocks, scalp
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how do you diagnose dermatitis herpetiformis?
- histology
- immunofluorescence
- IgA seen in dermal papillae
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what is Rx of dermatitis herpetiformis?
- gluten free diet
- +/- dapsone
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if you see targetoid blisters - what is that?
erythema multiforme
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what are causes of erythema multiform?
- infection - herpes, mycoplasma
- drugs: anticonvulsants - phenytoin, antibiotics - sulphonamides, NSAIDs
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what is the difference between EM and steven johnsons?
in stephen johnsons: mucous membrane involvement - eye and mouth
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what is the worst part of the spectrum of erythema multiform?
- toxic epidermal necrolysis
- dermatological emergency
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what is the usual cause of TEN?
drug induced
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how would you describe TEN?
- blistering
- haemorrhage
- necrosis - purpura
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what is the Rx of TEN?
- transfer to burns unit or ITU
- stop implicated drug
- fluid balance
- nutrition
- temperature
- prevent infection
- ivIG
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what causes porphyria cutanea tarda?
deficiency of enzyme needed in HAEM synthesis
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what are symptoms of porphyria cutanea tarda?
- photosensitivity's in the form of blisters and erosions on sun exposed skin areas
- hyperpigmentation of skin
- hypertrichosis especially on upper cheeks
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what other medical problem is porphyria cutanea tarda associated with?
liver problems
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what are Ix for porphyria cutanea tarda?
urine, blood, faeces analysis for porphyrins
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what is Rx of porphyria cutanea tarda?
- avoid alcohol, iron supplements, excess sun exposure
- low dose antimalarials
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