56 - Epidermolysis Bullosa Acquisita

Question 1

EBA is cause by IgG autoantibodies to

Answer 1

Type VII collagen

Question 2

Common sites of EBA are

Answer 2

Trauma-prone areas

Question 3

Mode of inheritance of EBA

Answer 3

Sporadic

Question 4

Both characterized by skin fragility, subepidermal blisters, milia formation and scarring; common of decreased anchoring fibrils

Answer 4

EBA

Hereditary forms of dystrophic EB

Question 5

Cause of decreases or absent anchoring fibrils is a genetic defect in the

Answer 5

COL7A1 gene

Question 6

Type VII collagen alpha chain domains

Answer 6

1. Noncollagenous 1 (NC-1) domain
2. Helical domain
3. Noncollagenous 2 (NC-2) domain

Question 7

Most EBA autoantibodies recognize 4 predominant antigenic epitopes within the _____ domain

Answer 7

NC-1

Question 8

When compared with BP, EBA patients had a higher presence of

Answer 8

C3b and C5

Question 9

Clinical presentations of EBA

Answer 9

1. Classic presentation
2. BP-like presentation
3. MMP-like presentation
4. Presentation reminiscent of Brunsting-Perry pemphigoid
5. Presentation reminiscent of LIGAD or CBDC

Question 10

Most common presentations of EBA

Answer 10

Classical

BP-like

Question 11

Noninflammatory bullous disease with an acral distribution that heals with scarring with milia formation

Answer 11

Classical presentation of EBA

Question 12

Classical presentation of EBA is reminiscent of ______ when mild and of the ______ when it is severe

Answer 12

Porphyria cutanea tarda

Hereditary form of recessive dystrophic EB

Question 13

Widespread, inflammatory vesiculobullous eruption involving the trunk, central body, and skin folds in addition to the extremities

Answer 13

BP-like presentation

Question 14

Predominant mucosal involvement with or without similar lesions on the glabrous skin

Answer 14

MMP-like presentation

Question 15

Chronic, recurrent vesiculobullous eruption localized to the head and neck and characterized by residual scars, subepidermal bullae, IgG deposits at the DEJ, and minimal or no mucosal involvement

Answer 15

Brunsting-Perry pemphigoid-like presentation

Question 16

Subepidermal bullous eruption, a neutrophilic infiltrate, and linear IgA deposits at the BMZ

Answer 16

IgA bullous dermatosis-like presentation

Question 17

Although mucosal involvement is frequent and severe in (childhood/adult) EBA, the overall prognosis is more favorable than in (childhood/adult) EBA

Answer 17

Childhood

Adult

Question 18

Systemic disease most frequently associated with EBA

Answer 18

Inflammatory bowel disease

Question 19

DIF of PCR vs EBA

Answer 19

Both have IgG and complement deposits at the DEJ

PCT - also demonstrates immune deposits around the dermal blood vessels

Question 20

EBA IIF substrate

Answer 20

Money or rabbit esophagus or human skin

Question 21

EBA have immune deposits with the _____ zone of the cutaneous BMZ

Answer 21

Sublamina densa

Question 22

Salt-split skin: Serum IgG on the epidermal roof

Answer 22

BP

Question 23

Salt-split skin: Serum IgG on the dermal side

Answer 23

EBA

Bullous SLE

Question 24

Y/N: EBA usually responds poorly to treatment

Answer 24

Yes

Question 25

EBA patients are often refractory to high doses of systemic glucocorticoids, azathioprine, methotrexate, and cyclophosphamide, especially when they have the ______ form of the disease

Answer 25

Classic mechanobullous form

Question 26

Often used as a first-line drug for EBA

Answer 26

Colchicine

Question 27

Common side effect of colchicine

Answer 27

Diarrhea