Bullous Disease

Question 1

bullous disease are…

Answer 1

autoimmune

Question 2

what are the 3 bullous diseases?

Answer 2

pemphigus vulgaris, bullous pemphigoid, dermatitis herpetiformis

Question 3

what is pemphigus vulgaris?

Answer 3

life-threatening blistering disease

Question 4

epi of pemphigus vulgaris

Answer 4

mid adult life

Question 5

pathophysiology of pemphigus vulgaris

Answer 5

IgG4 Abs against desmosomal proteins cause loss of keratinocyte adhesion in the skin and mucous membranes leading to superficial blistering, erosions, ulcers

Question 6

histology of pemphigus vulgaris

Answer 6

superficial split!- separation of individual keratinocytes

Question 7

common areas of PV

Answer 7

scalp, face, axilla, groin

Question 8

s/s of PV?

Answer 8

oral ulceration, non-itchy flaccid blisters- fluid filled, blisters may rupture leaving erythematous weeping erosions

Question 9

ix for PV?

Answer 9

biopsy & direct immunofluorescence of perilesional skin, Nikolsky’s sign

Question 10

what is Nikolsky’s sign

Answer 10

skin rubs off easily

Question 11

mx of PV?

Answer 11

systemic steroids, tetracycline, emollients etc

immunosuppression in long disease

Question 12

prognosis of PV?

Answer 12

chronic self-limiting course, remission achieved between 3-6months

Question 13

epi of bullous pemphigoid

Answer 13

more common than pemphigus, affects >60s

Question 14

pathophysiology of BP?

Answer 14

deeper split! local compliment activation (auto IgGs) against hemidesmosomal protein causing split through DEJ

Question 15

s/s of BP?

Answer 15

large tense serous/ haemorrhagic blisters, itchy

no mucosal involvement

Question 16

sites of BP?

Answer 16

localised, trunk, proximal limbs

Question 17

histology of BP?

Answer 17

sup-epidermal split and IgG compliment at DEJ

Question 18

ix for BP?

Answer 18

biopsy

Question 19

mx of BP?

Answer 19

oral corticosteroids & steroid sparing agents

Question 20

morbidity of BP?

Answer 20

self limiting but may take months to years to resolve

Question 21

what is dermatitis herpetiformis

Answer 21

a rare blistering condition associated with gluten sensitive enteropathy

Question 22

epi of dermatitis herpetiformis

Answer 22

M>F, early adult life

Question 23

aetiology of dermatitis herpetiformis ?

Answer 23

associated with coeliac

Question 24

s/s of dermatitis herpetiformis

Answer 24

small, intensely itchy blisters with papules

Question 25

sites of dermatitis herpetiformis

Answer 25

elbows, extensor forearms, scalp and buttocks

Question 26

ix for dermatitis herpetiformis

Answer 26

biopsy- sub-epi blistering + neutrophil micro abscesses in dermal papillae

Question 27

mx of dermatitis herpetiformis

Answer 27

avoid gluten and dapsone/ sulphonamides for symptom relief