58 - Linear Immunoglobulin A Dermatosis and Chronic Bullous Disease of Childhood

Question 1

Drug-induced linear IgA is associated with

Answer 1

Vancomycin

Question 2

Linear IgA dermatosis onset typically after

Answer 2

Fourth decade of line

Question 3

Chronic bullous disease of childhood presenting predominantly in

Answer 3

Children younger than 5 years of age

Question 4

Subclass of IgA found in the skin of LIGAD/CBDC

Answer 4

IgA1

Question 5

IgA subclass most often associated with mucosa

Answer 5

IgA2

Question 6

Antigenic targets in LIGAD/CBDC

Answer 6

Multiple antigens may be involved

Question 7

Pruritus: LIGAD vs DH

Answer 7

LIGAD - less severe than that seen in patients with DH

Question 8

Tense bullae, often on an inflammatory base most frequently in the perineum and perioral region, giving a “cluster of jewels” appearance

Answer 8

CBDC

Question 9

Relative paucity of serious mucosal involvement: LIGAD vs CBDC

Answer 9

CBDC

Question 10

Good prognosis: LIGAD vs CBDC

Answer 10

CBDC

Question 11

Mucosal involvement appears to be (more/less) prominent in patients with drug-induced linear IgA

Answer 11

Less

Question 12

Most common nonimmediate hypersensitivity reaction to vancomycin

Answer 12

LIGAD

Question 13

In LIGAD/CBDC, the blister forms

Answer 13

Within the lamina lucida or in a sublamina densa location

Question 14

Histopathology: LIGAD vs DH

Answer 14

LIGAD - fewer papillary microabscesses and a more diffuse infiltrate of neutrophils at the BMZ

Question 15

Y/N - histopathology off blisters in LIGAD, CBDC and DH is virtually indistinguishable

Answer 15

Yes

Question 16

DIF: LIGAD vs DIH

Answer 16

LIGAD - linear IgA at the BMZ

DH - granular IgA at the BMZ

Question 17

Chief physical trigger of LIGAD

Answer 17

UV light

Question 18

Disease associated with LIGAD

Answer 18

Possible association with ulcerative colitis

Question 19

Response to dapsone or sulfapyridine: LIGAD vs DH

Answer 19

Response usually occurs within 24-48 hours for both; not a helpful diagnostic sign

Question 20

CBDC course

Answer 20

Self-limiting disease, with most children going into remission within 2 years