Blistering Disease Review Qs Part 2

Question 1

collagen that attaches type 1 and 3 collagen to the lamina densa

Answer 1

type 7 collagen (anchoring fibrils)

Question 2

predominant collagen in lamina densa

Answer 2

type 4 collagen

Question 3

anchoring fibrils location

Answer 3

sublamina densa

Question 4

anchoring filaments location

Answer 4

lamina lucida

Question 5

5 members of the plakin flamily

Answer 5

desmoplakin
envoplakin
periplakin
plectin
BPAg 1

Question 6

2 cadherins in the skin

Answer 6

desmoglein and desmocollin

Question 7

diseases affecting collagen 7

Answer 7

Epidermolysis bullosa acquisita
dystrophic epidermolysis bullosa
bullous SLE
cicatricial pemphigoid

Question 8

diseases with immunofluorescence on base of salt split skin

Answer 8

anti-epilegrin cicatricial pemphigoid
epidermolysis bullosa aquisita
bullous LE

Question 9

diseases with immunofluorescence on roof of salt split skin

Answer 9

bullous pemphigoid
pemphigoid gestationis
linear IgA bullous dermatosis
cicatricial pemphigoid

Question 10

epidermolysis bullosa simplex with muscular dystrophy antigen

Answer 10

plectin

Question 11

junctional epidermolysis bullosa with pyloric atresia antigen

Answer 11

alpha 6 beta 4 integrin

Question 12

cicatricial pemphigus antigens (4)

Answer 12

BPAg 1 & 2, LAM 5 & 6

Question 13

herpes gestationis antigen

Answer 13

BPAg 2 (NC16A domain)

Question 14

BPAg 2 97-120 kDa antigen disease

Answer 14

linear IgA ds

Question 15

bullous pemphigoid antigens

Answer 15

BPAg 1 (BP230) - intermediate filaments to HD plaque
BPAg2 (BP180) - transmembrane protein attaches to laminin 5