Linear IgA bullous dermatosis/chronic bullous disease of childhood

Question 1

Ages affected by linear IgA bullous dermatosis/chronic bullous disease of childhood?

Answer 1

• elderly ( >60) and preschool-aged children (average age 4)

Question 2

Adult onset linear IgA bullous dermatosis is caused by_____

Answer 2

drug induced

Question 3

Most common drug causing Adult onset linear IgA bullous dermatosis?

Answer 3

Vancomycin

Question 4

Pathogenesis of LABD/CBDC (linear IgA dermatosis)

Answer 4

• IgA autoantibodies directed against two antigens derived from BPAG2:

- LAD-1

- LABD97

Question 5

What are the antigens targeted by IgA autoantibodies in LABD/CBDC?

Answer 5

- LAD-1

- LABD97

Question 6

Describe clinical presentation of LABD/CBDC.

Answer 6

• tense vesicles/bullae and urticarial plaques in annular, polycyclic, or herptiform (crown of jewels) arrangement

Question 7

Most common site affected in LABD/CBDC?

Answer 7

• flexures of lower trunk/thigh/groin/buttocks and face in kids

Question 8

What can you see at peripheral edge of lesiosn of LABD/CBDC?

Answer 8

• vesiculopustules at expanding edge of plaques

Question 9

How can you distinguish LABD/CBDC from DH?

Answer 9

• need DIF (histopath cannot distinguish)

Question 10

Histopath of LABD/CBDC?

Answer 10

• neutrophils diffusely lined up along BMZ (early lesions)
• subepidermal blister w/ neutrophils inside cavity (fully developed lesions

Question 11

DIF of LABD/CBDC will show?

Answer 11

• linear IgA along BMZ

Question 12

Treatment of choice for LABD/CBDC?

Answer 12

• Dapsone leads to rapid response in 72 hours (remember that this is histologically similar to DH and tx is the same)