title: Glucocorticosteroid-resistant pemphigoid gestationis: successful treatment with adjuvant immunoadsorption.
abstract: A 40-year old prima para presented with multiple urticaria-like plaques and severe pruritus 2 weeks prior to giving birth by cesarean section. Three days after birth, the disease flared up and tense blisters appeared on hands, lower arms and feet. Based on the clinical presentation, direct immunofluorescence microscopy, complement binding test and detection of high levels of circulating anti-BP180 antibodies, the diagnosis of pemphigoid gestationis was established. Despite treatment with class IV topical corticosteroid and prednisolone p.o. up to 60 mg/day, both skin lesions and severe pruritus progressed accompanied by increasing anti-BP180 antibody serum levels. In order to continue breast feeding, the prednisolone dose could not be further increased and 10 immunoadsorptions over 4 weeks were performed. During this period, skin lesions cleared rapidly, pruritus subsided and BP180-specific serum autoantibodies decreased by 99.5% allowing the reduction of prednisolone to 7.5 mg/day. We conclude that immunoadsorption is an effective and safe adjuvant therapeutic option for severe pemphigoid gestationis.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Lilia
authors.initials: L
authors.lastname: Westermann
authors.forename: Rainer
authors.initials: R
authors.lastname: Hügel
authors.forename: Markus
authors.initials: M
authors.lastname: Meier
authors.forename: Michael
authors.initials: M
authors.lastname: Weichenthal
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.forename: Regine
authors.initials: R
authors.lastname: Gläser
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
mesh_terms: D000328:Adult; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D004351:Drug Resistance; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006680:HLA Antigens / Q000276:immunology; D006801:Humans; D007163:Immunosorbent Techniques; D024041:Non-Fibrillar Collagens / Q000276:immunology; D006559:Pemphigoid Gestationis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000628:therapy*; D011239:Prednisolone / Q000627:therapeutic use; D011247:Pregnancy; D016896:Treatment Outcome; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D005938:Glucocorticoids; D006680:HLA Antigens; D024041:Non-Fibrillar Collagens; D011239:Prednisolone; D000097805:Collagen Type XVII