title: Serum levels of autoantibodies to desmoglein 3 in patients with therapy-resistant pemphigus vulgaris successfully treated with adjuvant intravenous immunoglobulins.
abstract: The mainstay of treatment of pemphigus vulgaris is systemic corticosteroids. Intravenous immunoglobulins have been reported as an adjuvant corticosteroid-sparing regimen in recalcitrant pemphigus vulgaris. The purpose of the study was to monitor disease activity, serum levels of autoantibodies and doses of oral corticosteroids in 4 patients with recalcitrant pemphigus vulgaris adjuvantly treated with intravenous immunoglobulins (2 g kg(-1) monthly). After initiation of intravenous immunoglobulins, all patients showed clinical improvement and a decrease in autoantibody serum levels, as detected by both indirect immunofluorescence microscopy and ELISA. Corticosteroids and immunosuppressants could be reduced and even discontinued in one patient. In 3 patients, intravenous immunoglobulins were discontinued after 12 cycles. Subsequently, new blisters developed and autoantibody levels rose again. After re-initiation of intravenous immunoglobulins, in 2 patients, the condition quickly improved again, along with a decrease in autoantibody serum levels. It is concluded that the administration of intravenous immunoglobulins was associated with a decrease in circulating autoantibodies and clinical improvement in our patients.
authors.affiliation: Department of Dermatology, University of Würzburg, Würzburg, Germany.
authors.forename: Susanne
authors.initials: S
authors.lastname: Herzog
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.forename: Matthias
authors.initials: M
authors.lastname: Goebeler
authors.forename: Eva-B
authors.initials: EB
authors.lastname: Bröcker
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
mesh_terms: D000284:Administration, Oral; D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D015820:Cadherins / Q000276:immunology*; D051184:Desmoglein 3; D004351:Drug Resistance; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology* / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D015820:Cadherins; C071894:DSG3 protein, human; D051184:Desmoglein 3; D016756:Immunoglobulins, Intravenous