Chapter 59 Pemphigoid Gestationis Flashcards
associated in babies w/ slight increase
in premature and small for gestational age births
during what part of pregnancy
later part or immediate postpartum
anti BMZ antibody (formerly called HG factor or IgG) induces ___ deposition
C3
nearly all have antibodies to
BP180 (type XVII collagen)
BP 230 coded for on the short arm of
chromosome 6
BP180 coded on long arm of
chromosome 10
Increase in antigens?
DR3 and DR4
Only source of disparate HLA antigens is typically the ?
placenta (primarily paternal)
autoantibody of gestational pemphigoid binds to ?
amniotic BM
from fetal ectoderm, antigenically similar to skin
disease initiated by aberrant expression of
MHC calss II antigens (paternal haplotype) w/ in placenta
associated w/ what disease?
H moles and choriocarcinoma
occurs during _____ pregnancy
late
first onset on which area of the body?
abdomen often within or immediately adjacent to umbilicus
rapid progression to a generalized pemphigoid-like eruption sparing the ___
face, mucous membranes, palms and soles is the rule
flares occur during which part of pregnancy?
delivery (approx 75%)
newborns may be affected up to __% of the time
10%
mild and self-limited
differentiate BP from PG
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
typical lesions of PG
urticarial or arcuate plaques rapidly progress toward mixed dermatitis including tense pemphigoid like blisters
Histopathology
subepidermal vesicle w/ lymphocytes and eosinophils
most constant histologic feature of PG
eosinophils
special test show
C3 w/ or w/o IgG along BMZ of perilesional skin
salt split skin specimen shows
antibody deposition along bottom of epidermal fragment (similar in BP)
Increased incidence of _______ antibodies documented in those w/ history of PG
antithyroid antibodies (clinically apparent thyroid dysfunction rare)
differential diagnosis
urticarial pemphigoid gestationis
PUPPP
Contact dermatitis (ACD)
drug eruption
complications in newborns 1)____ and 2)____
cutaneous disease - self limited, rarely requires intervention
increased risk of premature, small for GA
women w/ history of PG have increased risk for
Graves disease
course and prognosis
many have spontaneous resolution during later part of gestation only to experience flare at delivery
frequency of skip pregnancies approximates ___%
5-10%
recurrences w/ ____
flares during subsequent use of ____
menstruation
OCP
cornerstone of therapy
systemic CCS
dose of prednisone
0.5mg/kg daily
alternative treatment
dapsone
pyridoxine
cyclosporine
rituxan
