title: Cicatricial pemphigoid with circulating IgA and IgG autoantibodies to the central portion of the BP180 ectodomain: beneficial effect of adjuvant therapy with high-dose intravenous immunoglobulin.
abstract: Cicatricial pemphigoid (CP) is an autoimmune subepidermal blistering disease characterized by deposits of IgG, IgA, or C3 at the cutaneous basement membrane zone. CP may present with considerable variation regarding age, morphology of lesions, and mucosal involvement, which may heal with or without scarring. We describe a patient with CP who presented with circulating IgA and IgG autoantibodies to the epidermal side of salt-split human skin. By immunoblot analysis, the patient's IgA reacted with the soluble ectodomain of BP180 (LAD-1). This reactivity was mainly directed to the central portion of the BP180 ectodomain, a site that, to date, has not been described as the target of IgA autoantibodies. Different immunosuppressive treatment regimens including steroids and mycophenolate mofetil did not control this patient's disease, and severe scarring of the conjunctivae occurred with impairment of vision. Addition of adjuvant intravenous immunoglobulin (1 g/kg body weight on 2 consecutive days) every 4 weeks led to a dramatic improvement of conjunctivitis and gingivitis. Clinical improvement correlated with the serum's IgA immunoblot reactivity against LAD-1. Further studies on a larger number of patients with CP should try to correlate the specificity of autoantibodies in CP with the response to certain therapeutic regimens.
authors.affiliation: Department of Dermatology, University of Würzburg, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Leverkus
authors.forename: Matthias
authors.initials: M
authors.lastname: Georgi
authors.forename: Zhuxiang
authors.initials: Z
authors.lastname: Nie
authors.forename: Takashi
authors.initials: T
authors.lastname: Hashimoto
authors.forename: Eva-Bettina
authors.initials: EB
authors.lastname: Bröcker
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000276:immunology*; D001324:Autoantigens; D001485:Basement Membrane / Q000276:immunology; D029742:Caenorhabditis elegans Proteins; D015816:Cell Adhesion Molecules, Neuronal; D003231:Conjunctivitis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D018604:Epitope Mapping; D005260:Female; D005455:Fluorescent Antibody Technique; D005891:Gingivitis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D015801:Helminth Proteins; D006801:Humans; D015151:Immunoblotting; D007070:Immunoglobulin A / Q000276:immunology*; D007074:Immunoglobulin G / Q000276:immunology*; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D019006:Neural Cell Adhesion Molecules; D024041:Non-Fibrillar Collagens; D010390:Pemphigoid, Benign Mucous Membrane / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D029742:Caenorhabditis elegans Proteins; D015816:Cell Adhesion Molecules, Neuronal; D015801:Helminth Proteins; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D019006:Neural Cell Adhesion Molecules; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C501528:SAX-7 protein, C elegans