Derm- blistering skin disorders

Question 1

what is a bullae

Answer 1

large, fluid filled blister on the skin

Question 2

what is bullous pemphigoid

Answer 2

subepidermal blister caused by an autoimmune reaction

Question 3

how old are patients typically who have bullous pemphigoid

Answer 3

elderly (>60)

Question 4

which type of hypersensitivity reaction is bullous pemphigoid

Answer 4

type II

Question 5

is bollous pemphigoid nikolsky sign positive or negative?

Answer 5

negative

Question 6

in bollous pemphigoid- Which antibodies react with the antigen of hemidesmosomes that anchor basal cells to the basement membrane?

Answer 6

anti-hemimesmosome antibodies (igG)

Question 7

main investigation for bollous pemphigoid

Answer 7

biopsy- immunofluorescence

Question 8

management of localised bullous pemphigoid

Answer 8

High potency topical steroids at onset of symptoms e.g. itch

Question 9

what is pemphigus vulgaris

Answer 9

One of the autoimmune blistering skin conditions characterised by flaccid intra-epidermal blisters

Question 10

when does pemphigus vulgaris usually present

Answer 10

middle age

Question 11

drug triggers of pemphigus vulgaris

Answer 11

ACEi
penicillamine

Question 12

which type of hypersensitivity reaction is pemphigus vulgaris

Answer 12

type II

Question 13

is nikolsky sign positive or negative with pemphigus vulgaris

Answer 13

positive

Question 14

common sites for pemphigus vulgaris

Answer 14

mocosal surfaces
face
scalp
axillae
groin
trunk

Question 15

biopsy Immunofluorescence shows what in pemphigus vulgaris?

Answer 15

IgG in “chicken wire” pattern

Question 16

biopsy Immunofluorescence shows what in bullous pemphigoid?

Answer 16

linear band of IgG

Question 17

which antibodies are likely to be present in a patient with bullous pemphigoid

Answer 17

Anti-BP180 and anti-BP230 antibodies

Question 18

what is dermatitis herpetiformis

Answer 18

autoimmune bullous disorder associated with coeliac disease

Question 19

pathophysiology of dermatitis hereptiformis

Answer 19

igA antibodies cross react with TTG

Question 20

biopsy Immunofluorescence shows what in dermatitis herpetiformis?

Answer 20

granular deposits of IgA in dermal papillae

Question 21

drug used to treat dermatitis herpetiformis

Answer 21

dapsone

Question 22

pemphigUS and pemphiguS =

Answer 22

epidermUS
Superficial

Question 23

pemphigoiD =

Answer 23

Deep

Question 24

what type of hypersensitivity reaction is dermatitis herpetiformis

Answer 24

type III

Question 25

what is Stevens-johnson syndrome

Answer 25

immune-complex-mediated hypersensitivity disorder. ranges from mild-severe with TEN being most severe form

Question 26

predominant cause of SJS

Answer 26

adverse drug reaction

Question 27

drugs that are most common cause of SJS adverse drug reactions

Answer 27

sulfonamides beta-lactams (penicillins and cephalosporins) antiepeleptics allopurinol NSAIDs

Question 28

triggers of SJS

Answer 28

drug reactions infections- HSV, HIV, influenza, Epstein barr virus, hepatitis bacterial and fungal infections less common

Question 29

Nikolsky sign positive/negative in SJS

Answer 29

positive

Question 30

what is seen in at least two places with SJS

Answer 30

mucosal ulceration

Question 31

mortality rate in SJS

Answer 31

10%, 30% in TEN

Question 32

what is the definitive investigation to diagnose Stevens-johnson syndrome

Answer 32

skin biopsy

Question 33

what shape are the lesions seen in SJS

Answer 33

target shaped

Question 34

what is erythema multiforme

Answer 34

immune-mediated condition characterised by typical or atypical target lesions and potential blistering

Question 35

most common cause of erythema multiforme

Answer 35

infections- most common hsv

Question 36

which cell mediates erythema multiforme

Answer 36

delayed T cell mediated skin reaction

Question 37

what is erythema multiforme minor

Answer 37

does not involve mucous membranes usually triggered by an infection

Question 38

what is erythema multiforme major

Answer 38

does involve mucous membranes more commonly drug-induced

Question 39

how many colours are typical erythema multiforme lesions

Answer 39

three

Question 40

how many colours are atypical erythema multiforme lesions

Answer 40

two

Question 41

where do erythema multiforme rashes usually develop before spreading to rest of body

Answer 41

palms and soles

Question 42

what can be characteristic of the itch that occurs in bullous pemphigoid

Answer 42

may occur weeks or months before the appearance of any visible skin lesion