Chapter 59 Pemphigoid Gestationis

Question 1

associated in babies w/ slight increase

Answer 1

in premature and small for gestational age births

Question 2

during what part of pregnancy

Answer 2

later part or immediate postpartum

Question 3

anti BMZ antibody (formerly called HG factor or IgG) induces ___ deposition

Answer 3

C3

Question 4

nearly all have antibodies to

Answer 4

BP180 (type XVII collagen)

Question 5

BP 230 coded for on the short arm of

Answer 5

chromosome 6

Question 6

BP180 coded on long arm of

Answer 6

chromosome 10

Question 7

Increase in antigens?

Answer 7

DR3 and DR4

Question 8

Only source of disparate HLA antigens is typically the ?

Answer 8

placenta (primarily paternal)

Question 9

autoantibody of gestational pemphigoid binds to ?

Answer 9

amniotic BM

from fetal ectoderm, antigenically similar to skin

Question 10

disease initiated by aberrant expression of

Answer 10

MHC calss II antigens (paternal haplotype) w/ in placenta

Question 11

associated w/ what disease?

Answer 11

H moles and choriocarcinoma

Question 12

occurs during _____ pregnancy

Answer 12

late

Question 13

first onset on which area of the body?

Answer 13

abdomen often within or immediately adjacent to umbilicus

Question 14

rapid progression to a generalized pemphigoid-like eruption sparing the ___

Answer 14

face, mucous membranes, palms and soles is the rule

Question 15

flares occur during which part of pregnancy?

Answer 15

delivery (approx 75%)

Question 16

newborns may be affected up to __% of the time

Answer 16

10%

mild and self-limited

Question 17

differentiate BP from PG

Answer 17

BP - elderly, no gender bias
PG - strong association w/ HLA-DR3, -DR4, C4 null allele (not in BP)
PG - titer of anti-BMZ antibody usually so low (BP high)
BP - react to an intracellular 230-240kDa component of hemidesmosome while
PG - 180-kDa transmembrane protein

Question 18

typical lesions of PG

Answer 18

urticarial or arcuate plaques rapidly progress toward mixed dermatitis including tense pemphigoid like blisters

Question 19

Histopathology

Answer 19

subepidermal vesicle w/ lymphocytes and eosinophils

Question 20

most constant histologic feature of PG

Answer 20

eosinophils

Question 21

special test show

Answer 21

C3 w/ or w/o IgG along BMZ of perilesional skin

Question 22

salt split skin specimen shows

Answer 22

antibody deposition along bottom of epidermal fragment (similar in BP)

Question 23

Increased incidence of _______ antibodies documented in those w/ history of PG

Answer 23

antithyroid antibodies 
(clinically apparent thyroid dysfunction rare)

Question 24

differential diagnosis

Answer 24

urticarial pemphigoid gestationis
PUPPP
Contact dermatitis (ACD)
drug eruption

Question 25

complications in newborns 1)____ and 2)____

Answer 25

cutaneous disease - self limited, rarely requires intervention
increased risk of premature, small for GA

Question 26

women w/ history of PG have increased risk for

Answer 26

Graves disease

Question 27

course and prognosis

Answer 27

many have spontaneous resolution during later part of gestation only to experience flare at delivery

Question 28

frequency of skip pregnancies approximates ___%

Answer 28

5-10%

Question 29

recurrences w/ ____

flares during subsequent use of ____

Answer 29

menstruation

OCP

Question 30

cornerstone of therapy

Answer 30

systemic CCS

Question 31

dose of prednisone

Answer 31

0.5mg/kg daily

Question 32

alternative treatment

Answer 32

dapsone
pyridoxine
cyclosporine
rituxan