Blistering Dermatoses

Question 1

1st site of PV

Answer 1

Mouth (60%)> site of injury

Question 2

MC ab in PV

Answer 2

Dgl 3

Question 3

Mucocutaneous PV

Answer 3

Dgl 3 + 1

Question 4

Predominate dgl 3 ab

Answer 4

Mucosal lesions predominate

Question 5

PV pathophys

Answer 5

Plasminogen activator associated with ab mediated acantholysis

Th 2 ( but Th1 may be involved more in chronic

Question 6

PV associations

Answer 6

Myesthenia gravis and thymoma

Question 7

PV treatment

Answer 7

Steroids –> cellcept > azathioprine
Some consider rituximab first line
Continue meds until ab disappear then repeat DIF to predict remission

Question 8

Dominant autoimmune epitope in PV and PF

Answer 8

N-terminal extracellular domain of dsg 1

Question 9

Fogo selvagem

Answer 9

IL-1beta increased, strong Th1, IgM anti dsg 1

Question 10

% of pts with pemphigus erythematosus and pos lupus band

Answer 10

80%

Dsg 1, BP230, periplakin

Question 11

Definitive test in PNP

Answer 11

Immunoprecipitation reveals immune response against Desmoplakin, envoplakin, BPAG1, periplakin

Also dsg 1 and 3 - dsg 3 ab epitope spread broadly through the extracellular domain (vs PV)

IgG1 and 2 predominant (vs 4 in PV)

Question 12

HLA for PNP - significant

Answer 12

HLA-DRB1*03

Question 13

Ag in localized vulvar BP in young girls

Answer 13

230 kd ag

Question 14

% of BP with oral

Answer 14

20%

Question 15

LP pemphigoides ag

Answer 15

230, 180 and unique 200 kd

Question 16

Anti p-105 pemphigoid

Answer 16

Nonscarring, acute, widespread, severe MM involvement

DIF same as BP
Ag= 105 kd ag in lamina lucida

Question 17

% with positive circulating anti BM ab in BP

Answer 17

70%
Fix C (vs PV) which causes the damage

Question 18

Site of Ab binding

Answer 18

Lamina Lucida

IgG4

50% with eosinophilia

Question 19

% in BP with a positive DIF

Answer 19

100%, MC C’

Question 20

BP prognosis

Answer 20

Normally self limited over 5 years, less in kids

Question 21

Associated with increased risk of death in first yr of BP

Answer 21

Circulating 180 (but not 230)

Other RF- old age, high steroid dose at d/c, low albumin, and ESR >30

Question 22

Generalized inflammatory EBA

Answer 22

Associated diabetes and HLA-DR2

Question 23

Childhood DH

Answer 23

May also see palmar blisters and brown hemorrhagic purpuric macules

Question 24

Drug induced grovers

Answer 24

Cetuximab