Chapter 58 Linear IgA Dermatosis and CBDC

Question 1

Drug induced linear IgA initially described in association with?

Answer 1

Vancomycin

Question 2

Linear IgA occurs most often after _____ and after _____ ?

Answer 2

after puberty

after 4th decade

Question 3

in CBDC - increased frequency of human histocompatibility antigen __, __, __ ?

Answer 3

HLA-B8, -DR3, -DQ2

Question 4

Increased frequency of allele ___ in both adults and children w/ linear IgA

Answer 4

TNF2

Question 5

CBDC predominantly in what age

Answer 5

< 5 years old

Question 6

CBDC - tense bullae often in

Answer 6

perinuem and perioral region

“cluster of jewels” appearance

Question 7

IgA subclass in skin revealed almost exclusively ?

Answer 7

IgA1

Question 8

autoantibodies in linear IgA dermatosis recognize the soluble __?

Answer 8

120-kDa ectodomain of type XVII collagen

linear IgA, cicatricial P, BP

Question 9

antibodies of linear IgA directed against

Answer 9

NC16-A region of collagen type XVII

same as BP

Question 10

predominant antigenic target ?

Answer 10

BP 180 antigen (collagen XVII)

Some react w/ BP230, LAD 285

Question 11

Cutaneous findings

Answer 11

annular or grouped papules, vesicles and bullae

Question 12

Cutaneous findings in drug induced linear IgA

Answer 12

EM like findings and TEN like with widespread bullae

Question 13

most closely associated drug

Answer 13

vancomycin

Question 14

oral lesions ?

Answer 14

desquamative gingivitis and oral lesions consistent w/ cicatricial pemphigoid
(less often seen in CBDC and drug induced linear IgA)

Question 15

linear IgA associated w/ what condition?

Answer 15

UC and Crohn’s (abN mucosal IgA1 in UC)

Question 16

CBDC associated w/ ?

Answer 16

acute mononucleosis and Paecilomyces lung infection in chronic granulomatous disease

Question 17

most frequent inflammatory infiltrate and major component of subepidermal inflammation

Answer 17

neutrophils

Question 18

Electron microscopic exam of blisters - form either within ?

Answer 18

lamina lucida or in sublamina densa

Question 19

respond dramatically to what treatment?

Answer 19

dapsone or sulfapyridine

usually occurs w/ in 24-48 hrs

Question 20

unresponsive to dapsone and prednisone, Tx?

Answer 20

mycophenolate mofetil

Question 21

CBDC is self limited, most children going into remission within ?

Answer 21

2 years