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Gestational Pemphigoid: Placental Morphology and Function

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Gestational Pemphigoid: Placental Morphology and Function Acta Derm Venereol 2013; 93: 33–38 INVESTIGATIVE REPORT Gestational Pemphigoid: Placental Morphology and Function Laura HUILAJA1, Kaarin MÄKIKALLIO2, Raija SORMUNEN3, Jouko LOHI4, Tiina HURSKAINEN1 and Kaisa TASANEN1 1Department of Dermatology, and Oulu Center for Cell-Matrix Research, University of Oulu and Clinical Research Center, 2Department of Obstetrics and Gynecology, Oulu University Hospital, 3Biocenter Oulu, and Department of Pathology, University of Oulu, Oulu, and 4Department of Pathology, Haart- man Institute, University of Helsinki, Helsinki, Finland Gestational pemphigoid (PG), a very rare pregnancy- hemidesmosome complex mediating the adhesion of associated bullous dermatosis, is associated with adverse epithelial cells to the basement membrane (4–6). Colla- pregnancy outcome (miscarriage, preterm delivery, foe- gen XVII is predominantly expressed in skin, but is also tal growth restriction). The major antigen in PG is col- present in amniotic epithelia, placenta and amniotic fluid lagen XVII (BP180). PG autoantibodies cross-react with (7, 8). In PG, autoantibodies are mainly targeted against collagen XVII in the skin and have been suggested to the largest non-collagenous domain NC16A (9–12). The cause placental failure. On this basis, we evaluated clini- exact pathomechanism of PG remains unclear, but ab- cal outcome and morphological and functional placen- normal expression of MCH II (major histocompatibility tal data of 12 PG pregnancies in Finland during 2002 to complex) molecules in PG placenta has been suggested 2011. The placental-to-birth weight ratio was abnormal to trigger local allogeneic reaction against the feto-pla- in half of the pregnancies. Ultrastructural analysis of PG cental unit (13). PG autoantibodies are thought to cause placentas showed detachment of basement membranes placental failure and cross-react with collagen XVII in and undeveloped hemidesmosomes. Ultrasound evalua- skin (14, 15). In clinical practice, PG does not increase tions of placental function prior to delivery were normal foetal mortality, but an association with miscarriage, in all but one pregnancy. Three (25%) neonates were de- preterm delivery and foetal growth restriction has been livered preterm after 35 gestational weeks and one preg- reported (16–18). nancy was complicated by preeclampsia and severe foe- Due to a possible placental failure and the known asso- tal growth restriction. Neonatal outcome was uneventful ciation of PG with placenta-originated adverse pregnancy in every case. In conclusion, in pregnancies complicated outcomes, we wanted to evaluate morphological and by PG, slight alteration in ultrastructural morphology functional placental data as well as the clinical outcome of the placental basement membrane was detected, but of 12 PG pregnancies in Finland during 2002 to 2011. umbilical artery Doppler evaluation indicated no func- tional placental changes. Key words: pemphigoid gesta- tionis; BP180; collagen XVII; placenta; umbilical artery; MATERIALS AND METHODS ultrasound; pregnancy outcome. Patients (Accepted February 11, 2012.) Finnish dermatologists in all university and central hospitals were informed about this prospective PG study, which was Acta Derm Venereol 2013, 93: 33–38. approved by the ethics committee of Northern Ostrobothnia Hospital District, Oulu, Finland. Ten Finnish (Caucasian) Kaisa Tasanen, Department of Dermatology, University patients with 12 pregnancies were recruited, and an informed of Oulu, Aapistie 5A, FIN-90230 Oulu, Finland. E-mail: consent was requested after the confirmation of PG diagnosis [email protected] in the local units in 2002 to 2011. Inclusion criteria included typical cutaneous findings for PG and C3 positivity in direct immunofluorescence analysis of perilesional skin. Direct im- Pemphigoid gestationis (PG) is a rare pregnancy-asso- munofluorescence analysis of skin and placental (patient 7) samples were performed using standard procedures. Clinical ciated bullous dermatosis with an incidence of approxi- outcome data was collected from the patient records. The study mately 1:50,000 pregnancies (1). It typically manifests was performed according to the Declaration of Helsinki 1983 in the second and third trimesters, but may develop at and approved by the ethics committee of Northern Ostrobothnia any stage during pregnancy and puerperium. Clinically, Hospital District (reference number 61/2004). In addition, all PG is characterized by severe pruritus, urticarial papules, participating women gave informed consent for use of their medical data for research purposes. plaques and subsequent blister development. The diag- nostic hallmark of PG is linear C3 deposition along the Blood sampling and transmission electron microscopy of the basement membrane in the immunofluorescence staining placenta of perilesional skin. Concomitant IgG depositions and Circulating antibodies against collagen XVII were analysed in circulating serum IgG autoantibodies are also typical 6 out of 12 pregnancies by BP180-ELISA (Medical & Biolo- for PG (2, 3). The major antigen in PG is collagen XVII gical Laboratories, Nagoya, Japan) performed at HUSLAB in (BP180), which is a transmembrane component of the Helsinki, Finland. © 2013 The Authors. doi: 10.2340/00015555-1370 Acta Derm Venereol 93 Journal Compilation © 2013 Acta Dermato-Venereologica. ISSN 0001-5555 34 L. Huilaja et al. Transmission electron microscopic analysis of placental samp- Blisters were detected in the extremities, chest, back, les from 5 PG placentas and control placentas collected from areolas, vulva and armpits as well (Table I; see Fig. 1 uneventful pregnancies at Oulu University Hospital, Oulu, was performed by a single observer. The sample preparation for trans- for an example). Linear C3 depositions in the basement mission electron microscopy has been described earlier (19). membrane zone were present in all 12 cases. IgG de- positions were positive in 3 out of 12 (25%) women. Ultrasonographic placental examination Linear C3 depositions were also detected in the villous trophoblastic basement membrane zone in PG placenta Image-directed pulsed and colour Doppler ultrasound equipment (Acuson Sequoia 512; Acuson Corporation, Mountain View, (Fig. 2a), but not in normal placenta (data not shown). CA, USA; Voluson Expert 730 and Voluson E8, GE Medical At the onset of PG symptoms at 8–36 gestational weeks, Systems, Kretz, Austria) with 4–8 MHz convex probes was used anti-BP180-IgG levels were approximately 4–10-fold to obtain umbilical artery blood velocity waveforms from free compared with the upper limit of the normal value in loops of the umbilical cord. For the qualitative analysis of the all cases analysed (n = 6) (Table I). umbilical artery blood velocity waveforms, the high-pass filter was set to a minimum, and the angle of insonation was kept at Systemic corticosteroids were the mainstay treatment. < 15 degrees. The median interval between the ultrasonographic Prednisolone at doses of 20–50 mg per day was used examination and delivery was 5 days (range 0–21 days). in 8 out of 12 (66%) cases. Three women (25%) were treated successfully with topical corticosteroids and antihistaminic agents only, and all patients received RESULTS them in addition to systemic treatment. Cyclosporine Dermatological characteristics of the 12 included preg- was added to systemic corticosteroid due to severe PG nancies (10 women) are summarized in Table I. We manifestation in case 7. Four (33%) women continued have partly described patients 5 and 6 in our previous systemic corticosteroid treatment longer than one month study (8). after the delivery. Median (range) length of systemic cortisone treatment was 2 weeks (1–10) during preg- Clinical presentation nancy and 2 months (0.5–15) postpartum. Two (17%) women reported premenstrual flare-ups, and one of them All but 2 mothers with PG were multiparous. No PG still develops skin symptoms on her feet 6 years after was identified in their previous pregnancies (median the delivery. Two women have used oral contraceptives 2 (range 1–3)). The majority of the mothers (58%) and neither reported further symptoms of PG. presented symptoms during the last trimester of preg- nancy (Table I). Three mothers delivered after the index Prenatal testing and pregnancy outcome PG pregnancy, and PG developed in 2 (33%) of the 6 consecutive pregnancies. These 2 pregnancies were the Obstetric characteristics of the 12 PG pregnancies only ones in this series with PG onset during the first are presented in Table II. One pregnancy (8%) was trimester (Table I). Median postnatal follow-up was complicated by preeclampsia and foetal growth below 2.0 years (range 0.5–8.7 years). the 5th percentile (20). Three (25%) neonates were de- Pruritus was the principal symptom in all women. livered prematurely at > 35 gestational weeks with a At the first dermatological visit 7 (58%) women had spontaneous onset of labour. Antenatal evaluations of blistering eruptions and subsequently all but one de- foetal growth, biophysical profile score and Doppler veloped blisters. There was cutaneous involvement ultrasonographic evaluation of placental function were in the abdomen and periumbilical area in all cases. normal in all cases but one: foetal growth in the 2nd Table I. Demographic and clinical data of patients with gestational pemphigoid Pat. Age, GA at onset DIF findings Subsequent no. years of symtoms (C3/IgG) BP180-ELISAa Sites of involvement (including blisters) Main treatment pregnancy 1 35 24+ +/– 50 Abdomen, extremities
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