Immunobullous diseases

Question 1

Bullous Pemphigoid - what is it?

Answer 1

Chronic autoimmune bullous disorder

Question 2

Bullous Pemphigoid - who does it commonly affect?

Answer 2

Elderly people

Affects men and women equally

Question 3

Bullous Pemphigoid - pathogenesis

Answer 3

Subepidermal blister

Bullous pemphigoiD = split is DEEP through DE junction

Question 4

Bullous Pemphigoid - morphology

Answer 4

Large tense bullae
Blisters burst to form erosion
May first present with itchy erythematous plaques/papules
Urticated skin lesions

Question 5

Bullous Pemphigoid - clinical presentation

Answer 5

Itchy

Question 6

Bullous Pemphigoid - histology

Answer 6

Epiermis floats off of basement membrane

Predominance of eosinophils

Question 7

Bullous Pemphigoid - investigations

Answer 7

Immunofluorescence - IgG and C3 at basement membrane
Skin biopsy
Nikolsky sign = -ve

Question 8

Bullous Pemphigoid - common affected areas

Answer 8

Trunk and limbs

Question 9

Bullous Pemphigoid - management

Answer 9

High dose systemic corticosteroids

Topical steroids - in frail patients

Question 10

Pemphigus - what is it?

Answer 10

Rare autoimmune condition

Question 11

Pemphigus - who does it commonly affect?

Answer 11

Middle aged

Affects males and females equally

Question 12

Pemphigus - pathogenesis

Answer 12

PemphiguS - split is more SUPERFICIAL i.e. going through the epidermis
Loss of integrity of epidermal cell adhesion

Question 13

Pemphigus - morphology

Answer 13

Fragile fluid filled blisters
Blisters rupture to form shallow erosions
Can have mucosal involvement - in severe disease

Question 14

Pemphigus - common areas affected

Answer 14

Head

Torso

Question 15

Pemphigus - histology

Answer 15

Acantholysis (separation of epidermal cells)

Question 16

Pemphigus - investigations

Answer 16

Skin biopsy
Immunofluorescence
Nikolsky sign = +ve (indicates plane of cleavage is within the epidermis)

Question 17

Pemphigus - investigations

Answer 17

Skin biopsy
Immunofluorescence - IgG, desmoglian 3
Nikolsky sign = +ve (indicates plane of cleavage is within the epidermis)

Question 18

Pemphigus - management

Answer 18

High dose oral steroids
Life-long treatment
Immunosuppressants - azathioprine

Question 19

Epidermolysis bullosa - what is it?

Answer 19

Due to inherited skin abnormalities which result in skin fragility

Question 20

Epidermolysis bullosa - who does it commonly affect?

Answer 20

Infants (at birth or shortly after)

Question 21

Epidermolysis bullosa - 3 main types

Answer 21

Simplex
Junctional
Dystrophic

Question 22

Epidermolysis bullosa - morphology

Answer 22

Large fluid filled blisters

Eventually digits are lost an it results in mitten fingers

Question 23

Epidermolysis bullosa - clinical presentation

Answer 23

Extensive skin loss when baby is handled or moved

Question 24

Epidermolysis bullosa - pathogenesis

Answer 24

Mutation in one of the DE junction proteins causes the epidermis and dermis to separate from each other

Question 25

Epidermolysis bullosa - genetics

Answer 25

Haploinsufficiency

Question 26

Dermatitis Herpetiformis - what is it?

Answer 26

Uncommon

Subepidermal blistering eruption

Question 27

Dermatitis Herpetiformis - common associated disease?

Answer 27

Coeliac disease

Question 28

Dermatitis Herpetiformis - which gene is it associated with?

Answer 28

HLA-DQ2

Question 29

Dermatitis Herpetiformis - areas commonly affected

Answer 29

Buttocks
Elbows
Knees