58 - Linear Immunoglobulin A Dermatosis and Chronic Bullous Disease of Childhood Flashcards
Drug-induced linear IgA is associated with
Vancomycin
Linear IgA dermatosis onset typically after
Fourth decade of line
Chronic bullous disease of childhood presenting predominantly in
Children younger than 5 years of age
Subclass of IgA found in the skin of LIGAD/CBDC
IgA1
IgA subclass most often associated with mucosa
IgA2
Antigenic targets in LIGAD/CBDC
Multiple antigens may be involved
Pruritus: LIGAD vs DH
LIGAD - less severe than that seen in patients with DH
Tense bullae, often on an inflammatory base most frequently in the perineum and perioral region, giving a “cluster of jewels” appearance
CBDC
Relative paucity of serious mucosal involvement: LIGAD vs CBDC
CBDC
Good prognosis: LIGAD vs CBDC
CBDC
Mucosal involvement appears to be (more/less) prominent in patients with drug-induced linear IgA
Less
Most common nonimmediate hypersensitivity reaction to vancomycin
LIGAD
In LIGAD/CBDC, the blister forms
Within the lamina lucida or in a sublamina densa location
Histopathology: LIGAD vs DH
LIGAD - fewer papillary microabscesses and a more diffuse infiltrate of neutrophils at the BMZ
Y/N - histopathology off blisters in LIGAD, CBDC and DH is virtually indistinguishable
Yes
DIF: LIGAD vs DIH
LIGAD - linear IgA at the BMZ
DH - granular IgA at the BMZ
Chief physical trigger of LIGAD
UV light
Disease associated with LIGAD
Possible association with ulcerative colitis
Response to dapsone or sulfapyridine: LIGAD vs DH
Response usually occurs within 24-48 hours for both; not a helpful diagnostic sign
CBDC course
Self-limiting disease, with most children going into remission within 2 years
