Abstract
Pemphigoid gestationis is a rare autoimmune blistering disease of pregnancy involving autoantibodies targeting collagen XVII in the basement membrane zone of the skin. Patients usually present with pruritic urticarial papules and annular plaques eventually developing vesicles and tense bullae ranging from a few days to 4 weeks. The most common site for the lesions is the periumbilical area. Diagnosis is made by a high clinical suspicion on presentation and confirmed by histology and direct immunofluorescence. If required, indirect immunofluorescence, enzyme-linked immunosorbent assay, and immunoblotting may be done. Current management includes topical and systemic steroids, anti-inflammatory antibiotics, and immunosuppressives.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Black MM. The Neil Smith memorial lecture: John Laws Milton. The founder of St John’s Hospital for diseases of the skin. Clin Exp Dermatol. 2003;28:89–91.
Intong LRA, Murrell DF. Pemphigoid gestationis: pathogenesis and clinical features. Dermatol Clin. 2011;29:447–52.
Ambros-Rudolph CM, Müllegger RR, Vaughan-Jones SA, Kerl H, Black MM. The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients. J Am Acad Dermatol. 2006;54:395–404.
Shornick JK, Bangert JL, Freeman RG, Gilliam JN. Herpes gestationis: clinical and histologic features of twenty-eight cases. J Am Acad Dermatol. 1983;8:214–24.
Jenkins RE, Hern S, Black MM. Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol. 1999;24:255–9.
Semkova K, Black M. Pemphigoid gestationis: current insights into pathogenesis and treatment. Eur J Obstet Gynecol Reprod Biol. 2009;145:138–44.
Yip L, McCluskey J, Sinclair R. Immunological aspects of pregnancy. Clin Dermatol. 2006;24:84–7.
McPherson T, Venning VV. Management of autoimmune blistering diseases in pregnancy. Dermatol Clin. 2011;29:585–90.
Chi CC, Wang SH, Prenter A, Cooper S, Wojnarowska F. Basement membrane zone and dermal extracellular matrix of the vulva, vagina and amnion: an immunohistochemical study with comparison with non- reproductive epithelium. Australas J Dermatol. 2010;51:243–7.
Huilaja L, Hurskainen T, Autio-Harmainen H, Hofmann SC, Sormunen R, Räsänen J, et al. Pemphigoid gestationis autoantigen, transmembrane collagen XVII, promotes the migration of cytotrophoblastic cells of placenta and is a structural component of fetal membranes. Matrix Biol. 2008;27:190–200.
Di Zenzo G, Calabresi V, Grosso F, Caproni M, Ruffelli M, Zambruno G. The intracellular and extracellular domains of BP180 antigen comprise novel epitopes targeted by pemphigoid gestationis autoantibodies. J Invest Dermatol. 2007;127:864–73.
Shimanovich I, Skrobek C, Rose C, Nie Z, Hashimoto T, Bröcker EB, et al. Pemphigoid gestationis with predominant involvement of oral mucous membranes and IgA autoantibodies targeting the C-terminus of BP180. J Am Acad Dermatol. 2002;47:780–4.
Patton T, Plunkett RW, Beutner EH, Deng JS, Jukic DM. IgG4 as the predominant IgG subclass in pemphigoides gestationis. J Cutan Pathol. 2006;33:299–302.
Noe MH, Messingham KA, Brandt DS, Andrews JI, Fairley JA. Pregnant women have increased incidence of IgE antibodies reactive with the skin and placental antigen BP180 (type XVII collagen). J Reprod Immunol. 2010;85:198–204.
Martel P, Gilbert D, Drouot L, Prost C, Raux G, Delaporte E, et al. A polymorphic variant of the gene coding desmoglein 1, the target autoantigen of pemphigus foliaceus, is associated with the disease. Genes Immun. 2001;2:41–3.
Martel P, Gilbert D, Busson M, Loiseau P, Lepage V, Drouot L, et al. Epistasis between DSG1 and HLA class II genes in pemphigus foliaceus. Genes Immun. 2002;3:205–10.
García-González E, Castro-Llamas J, Karchmer S, Zúñiga J, de Oca DM, Ambaz M, et al. Class II major histocompatibility complex typing across the ethnic barrier in pemphigoid gestationis. A study in Mexicans. Int J Dermatol. 1999;38:46–51.
Shornick JK, Stastny P, Gilliam JN. High frequency of histocompatibility antigens HLA- DR3 and DR4 in herpes gestationis. J Clin Invest. 1981;68:553–5.
Nanda A, Al-Saeed K, Dvorak R, Al-Muzairai I, Al-Sabah H, Al-Arbash M, et al. Clinicopathological features and HLA tissue typing in pemphigoid gestationis patients in Kuwait. Clin Exp Dermatol. 2003;28:301–6.
Shornick JK, Jenkins RE, Briggs DC, Welsh KI, Kelly SE, Garvey MP, et al. Anti-HLA antibodies in pemphigoid gestationis (herpes gestationis). Br J Dermatol. 1993;129:257–9.
Da Silva JAP. Sex hormones and glucocorticoids: interactions with the immune system. Ann N Y Acad Sci. 1999;876:102–18.
Shornick JK. Herpes gestationis. J Am Acad Dermatol. 1987;17:539–56.
Holmes RC, Black MM. The specific dermatoses of pregnancy. J Am Acad Dermatol. 1983;8:405–12.
Chi CC, Wang SH, Charles-Holmes R, Ambros-Rudolph C, Powell J, Jenkins R, et al. Pemphigoid gestationis: early onset and blister formation are associated with adverse pregnancy outcomes. Br J Dermatol. 2009;160:1222–8.
Shornick JK, Black MM. Secondary autoimmune diseases in herpes gestationis (pemphigoid gestationis). J Am Acad Dermatol. 1992;26:563–6.
Cobo MF, Santi CG, Maruta CW, Aoki V, et al. Pemphigoid gestationis: clinical and laboratory evaluation. Clinics. 2009;64:1043–7.
Barnadas MA, Rubiales MV, González MJ, Puig L, García P, Baselga E, et al. Enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence testing in a bullous pemphigoid and pemphigoid gestationis. Int J Dermatol. 2008;47:1245–9.
Ghohestani R, Kanitakis J, Nicolas JF, Cozzani E, Claudy A, et al. Comparative sensitivity of indirect immunofluorescence to immunoblot assay for the detection of circulating antibodies to bullous pemphigoid antigens 1 and 2. Br J Dermatol. 1996;135:74–9.
Shornick JK, Black MM. Fetal risks in herpes gestationis. J Am Acad Dermatol. 1992;26:63–8.
Aoyama Y, Asai K, Hioki K, Funato M, Kondo N, Kitajima Y, et al. Herpes gestationis in a mother and newborn. Arch Dermatol. 2007;143:1168–72.
Intong LRA, Murrell DF. Pemphigoid gestationis. Dermatol Clin. 2011;29:621–8.
Castro LA, Lundell RB, Krause PK, Gibson LE. Clinical experience in pemphigoid gestationis: report of 10 cases. J Am Acad Dermatol. 2006;55:823–8.
Bedocs PM, Kumar V, Mahon MJ. Pemphigoid gestationis: a rare case and review. Arch Gynecol Obstet. 2009;279:235–8.
Lu PD, Ralston J, Kamino H, Stein JA. Pemphigoid gestationis. Dermatol Online J. 2010;16:10.
Gilbert C, Mazzotta P, Loebstein R, Koren G. Fetal safety of drugs used in the treatment of allergic rhinitis: a critical review. Drug Saf. 2005;28(8):707–19.
Einarson A, Bailey B, Jung G, Spizzirri D, Baillie M, Koren G. Prospective controlled study of hydroxyzine and cetirizine in pregnancy. Ann Allergy Asthma Immunol. 1997;78(2):183–6.
Kallen B. Use of antihistamine drugs in early pregnancy and delivery outcome. J Matern Fetal Neonatal Med. 2002;11(3):146–52.
Ito S, Blajchman A, Stephenson M, Eliopoulos C, Koren G. Prospective follow-up of adverse reactions in breast-fed infants exposed to maternal medication. Am J Obstet Gynecol. 1993;168(5):1393–9.
Chi CC, Myon-White RT, Wojnarowska FT. Safety of topical corticosteroids in pregnancy: a population-based cohort study. J Invest Dermatol. 2011;131:884–91.
Chi CC, Wang SH, Kirtschig G, Wojnarowska F. Systematic review of the safety of topical corticosteroids in pregnancy. J Am Acad Dermatol. 2010;62:694–705.
Little BB. Immunosuppressant therapy during gestation. Semin Perinatol. 1997;21:143–8.
Amato L, Coronella G, Berti S, Gallerani I, Moretti S, Fabbri P. Successful treatment with doxycycline and nicotinamide of two cases of persistent pemphigoid gestationis. J Dermatolog Treat. 2002;13:143–6.
Loo WJ, Dean D, Wojnarowska F. A severe persistent case of recurrent pemphigoid gestationis successfully treated with minocycline and nicotinamide. Clin Exp Dermatol. 2001;26:726–7.
Hern S, Harman K, Bhogal BS, Black MM. A severe persistent case of pemphigoid gestationis treated with intravenous immunoglobulins and cyclosporine. Clin Exp Dermatol. 1998;23:185–8.
Kreuter A, Harati A, Breuckmann F, Appelhans C, Altmeyer P. Intravenous immune globulin in the treatment of persistent pemphigoid gestationis. J Am Dermatol. 2004;51:1027–8.
Sereno C, Filipe O, Marques-Gomes M. Refractory herpes gestationis responsive to intravenous immunoglobulin: a case report. J Am Acad Dermatol. 2005;52(3 Suppl 1):116 [abstract: P1604].
Doiron P, Pratt M. Antepartum intravenous immunoglobulin therapy in refractory pemphigoid gestationis: case report and literature review. J Cutan Med Surg. 2010;14:189–92.
Nosten F, McGready R, d’Alessandro U, Bonell A, Verhoeff F, Menendez C, et al. Anti-malarial drugs in pregnancy: a review. Curr Drug Saf. 2006;1(1):1–15.
Thornton YS, Bowe ET. Neonatal hyperbilirubinemia after treatment with maternal leprosy. South Med J. 1989;82(5):668.
Cianchini G, Masini C, Lupi F, Corona R, De Pità O, Puddu P. Severe persistent pemphigoid gestationis: long-term remission with rituximab. Br J Dermatol. 2007;157:388–9.
Amato L, Mei S, Gallerani I, Moretti S, Fabbri P. A case of herpes gestationis: persistent disease or conversion to bullous pemphigoid. J Am Acad Dermatol. 2003;49:302–7.
Gisbert JP. Safety of immunomodulators and biologics for the treatment of inflammatory bowel disease during pregnancy and breast-feeding [review]. Inflamm Bowel Dis. 2010;16(5):881–95.
Alstead EM, Nelson-Piercy C. Inflammatory bowel disease in pregnancy. Gut. 2003;52(2):159–61.
Armenti VT, Ahlswede KM, Ahlswede BA, Jarrell BE, Moritz MJ, Burke JF. National Transplantation Pregnancy Registry–outcome of 154 pregnancies in cyclosporine-treated female kidney transplant recipients. Transplantation. 1994;57:502–6.
Armenti VT, Radomski JS, Moritz MJ, Gaughan WJ, Gulati R, McGrory CH, et al. Report from the National Transplantation Pregnancy Registry (NTPR): outcomes of pregnancy after transplantation. Clin Transpl. 2005:69–83.
Bar Oz B, Hackman R, Einarson T, Koren G. Pregnancy outcome after cyclosporine therapy during pregnancy: a meta-analysis. Transplantation. 2001;71:1051–5.
Castle SP, Mather-Mondrey M, Bennion S, David-Bajar K, Huff C. Chronic herpes gestationis and antiphospholipid antibody syndrome successfully treated with cyclophosphamide. J Am Acad Dermatol. 1996;34:333–6.
Gan DC, Welsh B, Webster M. Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum intravenous immunoglobulin followed by azathioprine. Australas J Dermatol. 2012;53:66–9.
Van de Wiel A, Hart HC, Flinterman J, Kerckhaert JA, Du Boeuff JA, Imhof JW. Plasma exchange in herpes gestationis. Br Med J. 1980;281:1041–2.
Marker M, Derfler K, Monshi B, Rappersberger K. Successful immunoapheresis of bullous autoimmune disease: pemphigus vulgaris and pemphigoid gestationis. J Dtsch Dermatol Ges. 2011;9:27–31.
Garvey MP, Handfield-Jones SE, Black MM. Pemphigoid gestationis-response to chemical oophorectomy with goserelin. Clin Exp Dermatol. 1992;17:443–5.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer-Verlag Berlin Heidelberg
About this chapter
Cite this chapter
Mendoza, C.G., Black, M.M., Murrell, D.F. (2015). Pemphigoid Gestationis. In: Murrell, D. (eds) Blistering Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-45698-9_32
Download citation
DOI: https://doi.org/10.1007/978-3-662-45698-9_32
Published:
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-662-45697-2
Online ISBN: 978-3-662-45698-9
eBook Packages: MedicineMedicine (R0)