Chi CC, Wang SH, Charles-Holmes R, et al. Pemphigoid gestationis: early onset and blister formation are associated with adverse pregnancy outcomes. Br J Dermatol. 2009;160(6):1222-8

Nuffield Department of Clinical Medicine, University of Oxford, UK.
British Journal of Dermatology (Impact Factor: 4.28). 03/2009; 160(6):1222-8. DOI: 10.1111/j.1365-2133.2009.09086.x
Source: PubMed


It is unclear whether clinical features of pemphigoid gestationis (PG), such as timing of onset and severity, may affect pregnancy outcomes or whether the adverse outcomes in pregnancies complicated by PG are related to or worsened by systemic corticosteroid treatment.
To evaluate the associations of adverse pregnancy outcomes with clinical features, autoantibody titre of PG, and systemic corticosteroid treatment.
We conducted a retrospective cohort study recruiting 61 pregnancies complicated by PG from the St John's Institute of Dermatology database which enrolled cases from dermatologists across the U.K., and two tertiary hospitals in the U.K. and Taiwan. Outcome measures included gestational age at delivery, preterm birth, birthweight, low birthweight (LBW, i.e. birthweight < 2500 g), small-for-gestational-age (i.e. birthweight below the 10th percentile for gestational age), fetal loss, congenital malformation, and mode of delivery.
After controlling for maternal age and comorbidity, decreased gestational age at delivery was significantly associated with presence of blisters (P = 0.017) and disease onset in the second trimester (P = 0.001). Reduced birthweight was significantly associated with disease onset in the first and second trimesters (P = 0.030 and 0.018, respectively) as was also LBW [adjusted odds ratio (95% confidence interval) 13.71 (1.22-154.59) and 10.76 (1.05-110.65), respectively]. No significant associations of adverse pregnancy outcomes with autoantibody titre or systemic corticosteroid treatment were found.
Onset of PG in the first or second trimester and presence of blisters may lead to adverse pregnancy outcomes including decreased gestational age at delivery, preterm birth, and LBW children. Such pregnancies should be considered high risk and appropriate obstetric care should be provided. Systemic corticosteroid treatment, in contrast, does not substantially affect pregnancy outcomes, and its use for PG in pregnant women is justified.

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    • "Fetal prognosis is generally good but there is an increase in prematurity and small-for-date babies. Only recently, it could be shown that this risk correlates with disease severity, as represented by early onset and blister formation, and not with corticosteroid treatment, as has been repeatedly speculated before18. Due to a passive transfer of antibodies from the mother to the fetus, about 10% of newborns may develop mild skin lesions which resolve spontaneously within days to weeks16. "
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    ABSTRACT: The specific dermatoses of pregnancy represent a heterogeneous group of pruritic skin diseases that have been recently reclassified and include pemphigoid (herpes) gestationis, polymorphic eruption of pregnancy (syn. pruritic urticarial papules and plaques of pregnancy), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. They are associated with severe pruritus that should never be neglected in pregnancy but always lead to an exact work-up of the patient. Clinical characteristics, in particular timing of onset, morphology and localization of skin lesions are crucial for diagnosis which, in case of pemphigoid gestationis and intrahepatic cholestasis of pregnancy, will be confirmed by specific immunofluorescence and laboratory findings. While polymorphic and atopic eruptions of pregnancy are distressing only to the mother because of pruritus, pemphigoid gestationis may be associated with prematurity and small-for-date babies and intrahepatic cholestasis of pregnancy poses an increased risk for fetal distress, prematurity, and stillbirth. Corticosteroids and antihistamines control pemphigoid gestationis, polymorphic and atopic eruptions of pregnancy; intrahepatic cholestasis of pregnancy, in contrast, should be treated with ursodeoxycholic acid. This review will focus on the new classification of pregnancy dermatoses, discuss them in detail, and present a practical algorithm to facilitate the management of the pregnant patient with skin lesions.
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    • "Twenty percent of infants born to women with PG develop transient blistering in the neonatal period due to transplacental passage of maternal autoantibodies (Shornick et al., 1983). These infants also tend to be small for gestational age (SGA), although increased mortality is not seen (Lawley et al., 1978; Ambros-Rudolf et al., 2006; Chi et al., 2009; Holmes and Black, 1984; Mascaro et al., 1995; Shornick and Black, 1992). Previous studies suggest the effects on the fetus are due to antibody accumulation in the placenta, resulting in placental insufficiency (Holmes and Black, 1984). "
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    ABSTRACT: BP180 (type XVII collagen) is a transmembrane protein expressed in a variety of cell types. It is also the target of autoantibodies in cutaneous autoimmune disease including bullous pemphigoid and pemphigoid gestationis, a disease unique to pregnancy. The purpose of this study was to determine the prevalence and specificity of cutaneous autoantibodies in a cohort of pregnant women. De-identified sera were collected from pregnant women (n=299) and from non-pregnant controls (n=134). Sera were analyzed by ELISA for the presence of IgG and IgE autoantibodies directed against several cutaneous autoantigens. IgE antibodies against the NC16A domain of BP180 were detected in 7.7% of pregnant women, compared to 2.2% of healthy controls (p=0.01). No increase in total or cutaneous autoantigen specific IgG was seen. Total serum IgE was within the normal range. Full-length BP180 was detected by western immunoblot in epidermal, keratinocyte, placental and cytotrophoblast (CTB) cell lysates. Furthermore, flow cytometry and indirect immunofluorescence confirmed the expression of BP180 on the surface of cultured CTBs. Finally, it was demonstrated that IgE antibodies in the pregnancy sera labeled not only cultured CTBs, but also the placental amnion and cutaneous basement membrane zone using indirect immunofluorescence. We conclude that some pregnant women develop antibodies specific for BP180, and that these autoantibodies are capable of binding both CTB and the placental amnion, potentially affecting placental function.
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