title: Oral pemphigoid autoantibodies preferentially target BP180 ectodomain.
abstract: Mucous membrane pemphigoid (MMP) comprises a heterogenous group of autoimmune subepithelial bullous diseases very frequently having oral involvement. Very few studies have investigated the immunological status of a subset of MMP, termed oral pemphigoid (OP), presenting with exclusive oral lesions. In this study we show that 75% of 20 OP patients without scarring phenotype possessed circulating autoantibodies against the BP180 molecule, indicating a prominent role of this protein as a target antigen in OP. Of note, the frequency of reactivity against BP180 ectodomain epitopes in OP was similar to that previously reported for MMP with cicatricial phenotype, while the lack of significant recognition of BP180 intracellular domain appears to characterize OP with respect to other diseases of the pemphigoid group. Finally, the combined use of sensitive techniques allowed the detection of circulating autoantibodies in 90% of OP patients, supporting the usefulness of this approach in the diagnosis of MMP disease.
authors.affiliation: Molecular and Cell Biology Laboratory, Istituto Dermopatico dell'Immacolata, IDI-IRCCS, Rome, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Calabresi
authors.forename: Marco
authors.initials: M
authors.lastname: Carrozzo
authors.forename: Emanuele
authors.initials: E
authors.lastname: Cozzani
authors.forename: Paolo
authors.initials: P
authors.lastname: Arduino
authors.forename: Giorgio
authors.initials: G
authors.lastname: Bertolusso
authors.forename: Federico
authors.initials: F
authors.lastname: Tirone
authors.forename: Aurora
authors.initials: A
authors.lastname: Parodi
authors.forename: Giovanna
authors.initials: G
authors.lastname: Zambruno
authors.forename: Giovanni
authors.initials: G
authors.lastname: Di Zenzo
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000378:metabolism*; D001324:Autoantigens / Q000276:immunology* / Q000378:metabolism*; D000939:Epitopes / Q000096:biosynthesis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa / Q000276:immunology* / Q000473:pathology; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000276:immunology* / Q000378:metabolism* / Q000473:pathology; D019151:Peptide Library; D017434:Protein Structure, Tertiary / Q000502:physiology; D012189:Retrospective Studies; D000097805:Collagen Type XVII
chemical_list: D001323:Autoantibodies; D001324:Autoantigens; D000939:Epitopes; D024041:Non-Fibrillar Collagens; D019151:Peptide Library; D000097805:Collagen Type XVII