Abstract
Pemphigoid gestationis (PG) is an autoimmune blistering disease that occurs during pregnancy or soon after delivery. It is possibly caused by cross-reactivity between placental antigens and collagen XVII in the skin. Patients typically present with an intensely pruritic, vesiculobullous rash that starts periumbilically and spreads outwards across the trunk and extremities. Treatment is with immunosuppressive agents; however, given that PG is very rare—estimated to occur in 1 in 50,000 pregnancies—there is a paucity of evidence around specific treatments. Treatment of PG is also complicated by the need to consider the health of the fetus. Often, evidence for the use of immunosuppressant medications in pregnant women for other diseases can be extrapolated to PG. We review the literature and present an algorithm for treatment in the pre- and post-partum periods for women with PG based on the evidence available. We also list areas of focus for the future.
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Abbreviations
- ABSIS:
-
Autoimmune Bullous Skin Disorder Intensity Score
- BP:
-
Bullous pemphigoid
- DIF:
-
Direct immunofluorescence
- ELISA:
-
Enzyme-linked immunosorbent assay
- IA:
-
Immunoadsorption
- IIF:
-
Indirect immunofluorescence
- LBW:
-
Low birth weight
- LHRH:
-
Luteinizing hormone releasing hormone
- PDAI:
-
Pemphigus Disease Area Index
- PG:
-
Pemphigoid gestationis
- TSH:
-
Thyroid stimulating hormone
References
Yancey KB. Herpes gestationis. Dermatol Clin. 1990;8(4):727–35.
Shornick JK, et al. Herpes gestationis: clinical and histologic features of twenty-eight cases. J Am Acad Dermatol. 1983;8(2):214–24.
Lehrhoff S, Pomeranz MK. Specific dermatoses of pregnancy and their treatment. Dermatol Ther. 2013;26(4):274–84.
Morganroth PA, McHargue CA. Vesicles and targetoid lesions in a postpartum woman. JAMA Dermatol. 2014;150(4):445–6.
Shornick JK. Dermatoses of pregnancy. Semin Cutan Med Surg. 1998;17(3):172–81.
Lipozencic J, Ljubojevic S, Bukvic-Mokos Z. Pemphigoid gestationis. Clin Dermatol. 2012;30(1):51–5.
Czernik A, et al. Intravenous immunoglobulin in the treatment of autoimmune bullous dermatoses: an update. Autoimmunity. 2012;45(1):111–8.
Chimanovitch I, et al. IgG1 and IgG3 are the major immunoglobulin subclasses targeting epitopes within the NC16A domain of BP180 in pemphigoid gestationis. J Invest Dermatol. 1999;113(1):140–2.
Intong LR, Murrell DF. Pemphigoid gestationis: pathogenesis and clinical features. Dermatol Clin. 2011;29(3):447–52, ix.
Roth MM. Pregnancy dermatoses: diagnosis, management, and controversies. Am J Clin Dermatol. 2011;12(1):25–41.
Yang B, et al. A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. Australas J Dermatol. 2014;55(1):e15–8.
Barnadas MA, et al. Enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence testing in a bullous pemphigoid and pemphigoid gestationis. Int J Dermatol. 2008;47(12):1245–9.
Intong LR, Murrell DF. Pemphigoid gestationis: current management. Dermatol Clin. 2011;29(4):621–8.
Rassai S, et al. Pemphigoid gestationis: a retrospective study in southwest Iran. Acta Med Iran. 2013;51(6):408–10.
Makol A, Wright K, Amin S. Rheumatoid arthritis and pregnancy: safety considerations in pharmacological management. Drugs. 2011;71(15):1973–87.
Pradat P, et al. First trimester exposure to corticosteroids and oral clefts. Birth Defects Res A Clin Mol Teratol. 2003;67(12):968–70.
Carmichael SL, et al. Maternal corticosteroid use and orofacial clefts. Am J Obstet Gynecol. 2007;197(6):585.e1–7; discussion 683–4, e1–7.
Braunstein I, Werth V. Treatment of dermatologic connective tissue disease and autoimmune blistering disorders in pregnancy. Dermatol Ther. 2013;26(4):354–63.
Asztalos EV, et al. Multiple courses of antenatal corticosteroids for preterm birth study: outcomes in children at 5 years of age (MACS-5). JAMA Pediatr. 2013;167(12):1102–10.
Chi CC, et al. Pemphigoid gestationis: early onset and blister formation are associated with adverse pregnancy outcomes. Br J Dermatol. 2009;160(6):1222–8.
Cobo MF, et al. Pemphigoid gestationis: clinical and laboratory evaluation. Clinics (Sao Paulo). 2009;64(11):1043–7.
Kreuter A, et al. Intravenous immune globulin in the treatment of persistent pemphigoid gestationis. J Am Acad Dermatol. 2004;51(6):1027–8.
Cianchini G, et al. Severe persistent pemphigoid gestationis: long-term remission with rituximab. Br J Dermatol. 2007;157(2):388–9.
Gisbert JP. Safety of immunomodulators and biologics for the treatment of inflammatory bowel disease during pregnancy and breast-feeding. Inflamm Bowel Dis. 2010;16(5):881–95.
Davison JM, Dellagrammatikas H, Parkin JM. Maternal azathioprine therapy and depressed haemopoiesis in the babies of renal allograft patients. Br J Obstet Gynaecol. 1985;92(3):233–9.
Amato L, et al. A case of chronic herpes gestationis: persistent disease or conversion to bullous pemphigoid? J Am Acad Dermatol. 2003;49(2):302–7.
Castle SP, et al. Chronic herpes gestationis and antiphospholipid antibody syndrome successfully treated with cyclophosphamide. J Am Acad Dermatol. 1996;34(2 Pt 2):333–6.
Rodrigues Cdos S, et al. Persistent herpes gestationis treated with high-dose intravenous immunoglobulin. Acta Derm Venereol. 2007;87(2):184–6.
Van de Wiel A, et al. Plasma exchange in herpes gestationis. Br Med J. 1980;281(6247):1041–2.
Westermann L, et al. Glucocorticosteroid-resistant pemphigoid gestationis: successful treatment with adjuvant immunoadsorption. J Dermatol. 2012;39(2):168–71.
Marker M, et al. Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis. J Dtsch Dermatol Ges. 2011;9(1):27–31.
Thomas S, et al. Postpartum pemphigoid gestationis. Indian J Dermatol. 2012;57(2):146–8.
Garvey MP, Handfield-Jones SE, Black MM. Pemphigoid gestationis – response to chemical oophorectomy with goserelin. Clin Exp Dermatol. 1992;17(6):443–5.
Daniel BS, et al. Severity score indexes for blistering diseases. Clin Dermatol. 2012;30(1):108–13.
Pfutze M, et al. Introducing a novel Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) in pemphigus. Eur J Dermatol. 2007;17(1):4–11.
Patsatsi A, et al. Association of autoantibodies to BP180 with disease activity in Greek patients with bullous pemphigoid. Clin Dev Immunol. 2012;2012:854795.
Murrell DF, et al. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol. 2008;58(6):1043–6.
Murrell DF, et al. Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts. J Am Acad Dermatol. 2012;66(3):479–85.
Loo WJ, Dean D, Wojnarowska F. A severe persistent case of recurrent pemphigoid gestationis successfully treated with minocycline and nicotinamide. Clin Exp Dermatol. 2001;26(8):726–7.
Amato L, et al. Successful treatment with doxycycline and nicotinamide of two cases of persistent pemphigoid gestationis. J Dermatolog Treat. 2002;13(3):143–6.
Shornick JK, Black MM. Fetal risks in herpes gestationis. J Am Acad Dermatol. 1992;26(1):63–8.
Tunzi M, Gray GR. Common skin conditions during pregnancy. Am Fam Physician. 2007;75(2):211–8.
Perez J, et al. Dyshidrosiform presentation (pompholyx-like) of pemphigoid gestationis with intrauterine fetal death. Int J Dermatol. 2013;53(11):1383–5.
Semkova K, Black M. Pemphigoid gestationis: current insights into pathogenesis and treatment. Eur J Obstet Gynecol Reprod Biol. 2009;145(2):138–44.
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This material is supported by the Veterans Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development.
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Moore, E.M., Werth, V.P. (2016). Pemphigoid Gestationis. In: Sami, N. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-26728-9_9
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DOI: https://doi.org/10.1007/978-3-319-26728-9_9
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