immunobullous disorders

Question 1

what is the primary feature of immunobullous disorders

Answer 1

blisters

Question 2

what are secondary features of immunobullous disorders and what causes them

Answer 2

vesicles and blisters - eczema, burns, herpes

Question 3

what are the main diseases

Answer 3

pemphigus vulgaris, bullous pemphigoid, dermatitis herpetiform

Question 4

what causes imunobullous disorders

Answer 4

autoimmune resulting in poor epidermal cell adhesion

Question 5

what are the investigations for immunobullous disorders

Answer 5

skin biopsy and immunofluorescence

Question 6

whats the main treatment for immunobullous disorders

Answer 6

steroids and immunosuppressant

Question 7

what is the pathology of pemphigus vulgaris

Answer 7

autoimmune against top layer of skin, IgG against desmoglein 3, immune complex forms, acatholysis

Question 8

what does desmoglein 3 do

Answer 8

maintains desmosome attachments

Question 9

what is acantholysis

Answer 9

loss if intercellular connections eg desmosomes

Question 10

where do blisters form in pemphigus vulgaris

Answer 10

intra-epidermal

Question 11

what are the blisters of pemphigus vulgaris like

Answer 11

multiple, painful, fragile blisters and erosions, superficial, nikolsky’s positive (top layer slides away from lower)

Question 12

where are you likely to get pemphigus vulgaris symptoms

Answer 12

scalp, face, axilla, eyes, mucous membranes eg mouth, GI, anus

Question 13

how do you manage pemphigus vulgaris

Answer 13

local steroids and analgesia, systemic: high dose steroids + immunosuppression (cycophosphamide/ azathioprine)

Question 14

who gets pemphigus vulgaris

Answer 14

middle aged people

Question 15

what is the pathology of bullous pemphigoid

Answer 15

IgG react with hemidesomosome basal cells in DEJ, local complement and tissue damage

Question 16

where do you get blisters in bullous pemphigoid

Answer 16

sub-epidermal at DEJ

Question 17

what are the blisters like in bullous pemphigoid

Answer 17

large, tense, itchy bullae, no acatholysis, nikolsys sign negative, no scarring, localised on trunk or proximal limb

Question 18

who gets bullous pemphigoid

Answer 18

elderly people

Question 19

how do you manage bullous pemphigoid

Answer 19

topical steroids - oral steroids +/- immunosuppressant +/- tetracycline +/– antihistamines

Question 20

what gene and disease is dermatitis herpetiformis associated with

Answer 20

HLADQ2, coeliac disease

Question 21

what are the symptoms of dermatitis herpetiformis

Answer 21

small, very itchy blisters on red base

Question 22

where are the blisters in dermatitis herpetiformis

Answer 22

elbows, knees, buttocks

Question 23

how do you diagnose dermatitis herpetiformis

Answer 23

immunofluorescence (papillary microabscess IgA), biopsy, bloods: antiTTG

Question 24

how do you manage dermatitis herpetiformis

Answer 24

gluten free +/- dapsone