Chapter 59 Pemphigoid Gestationis Flashcards

1
Q

associated in babies w/ slight increase

A

in premature and small for gestational age births

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2
Q

during what part of pregnancy

A

later part or immediate postpartum

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3
Q

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

A

C3

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4
Q

nearly all have antibodies to

A

BP180 (type XVII collagen)

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5
Q

BP 230 coded for on the short arm of

A

chromosome 6

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6
Q

BP180 coded on long arm of

A

chromosome 10

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7
Q

Increase in antigens?

A

DR3 and DR4

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8
Q

Only source of disparate HLA antigens is typically the ?

A

placenta (primarily paternal)

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9
Q

autoantibody of gestational pemphigoid binds to ?

A

amniotic BM

from fetal ectoderm, antigenically similar to skin

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10
Q

disease initiated by aberrant expression of

A

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

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11
Q

associated w/ what disease?

A

H moles and choriocarcinoma

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12
Q

occurs during _____ pregnancy

A

late

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13
Q

first onset on which area of the body?

A

abdomen often within or immediately adjacent to umbilicus

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14
Q

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

A

face, mucous membranes, palms and soles is the rule

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15
Q

flares occur during which part of pregnancy?

A

delivery (approx 75%)

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16
Q

newborns may be affected up to __% of the time

A

10%

mild and self-limited

17
Q

differentiate BP from PG

A

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

18
Q

typical lesions of PG

A

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

19
Q

Histopathology

A

subepidermal vesicle w/ lymphocytes and eosinophils

20
Q

most constant histologic feature of PG

A

eosinophils

21
Q

special test show

A

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

22
Q

salt split skin specimen shows

A

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

23
Q

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

A
antithyroid antibodies 
(clinically apparent thyroid dysfunction rare)
24
Q

differential diagnosis

A

urticarial pemphigoid gestationis
PUPPP
Contact dermatitis (ACD)
drug eruption

25
complications in newborns 1)____ and 2)____
cutaneous disease - self limited, rarely requires intervention increased risk of premature, small for GA
26
women w/ history of PG have increased risk for
Graves disease
27
course and prognosis
many have spontaneous resolution during later part of gestation only to experience flare at delivery
28
frequency of skip pregnancies approximates ___%
5-10%
29
recurrences w/ ____ | flares during subsequent use of ____
menstruation | OCP
30
cornerstone of therapy
systemic CCS
31
dose of prednisone
0.5mg/kg daily
32
alternative treatment
dapsone pyridoxine cyclosporine rituxan