title: The CD40/CD40 ligand system is involved in the pathogenesis of pemphigus.
abstract: The CD40/CD40 ligand (CD40L) system has never been investigated in autoimmune bullous diseases belonging to the pemphigus group in humans. Skin biopsy specimens from 21 patients with pemphigus vulgaris, 10 with pemphigus foliaceous and healthy volunteers were studied by immunohistochemistry (for CD40 and CD40L) and reversal transcriptase polymerase-chain reaction (for CD40L), while sera were analyzed by enzyme-linked immunosorbent assay for soluble CD40L. In all pemphigus specimens, the basal and suprabasal layers of the epidermis and perivascular infiltrating cells were CD40+. CD40L+ cells moderately infiltrated the perivascular and interstitial dermis of pemphigus specimens. CD40L mRNA was strongly evident in all pemphigus samples while no signal was detected in the healthy controls. The expression of soluble CD40L was significantly greater in pemphigus sera than in controls. We showed here that the CD40/CD40L system is upregulated both in lesional skin and in serum of patients with pemphigus.
authors.affiliation: Department of Dermatological Sciences, University of Florence, Florence, Italy.
authors.forename: Marzia
authors.initials: M
authors.lastname: Caproni
authors.forename: Emiliano
authors.initials: E
authors.lastname: Antiga
authors.forename: Daniele
authors.initials: D
authors.lastname: Torchia
authors.forename: Walter
authors.initials: W
authors.lastname: Volpi
authors.forename: Elena
authors.initials: E
authors.lastname: del Bianco
authors.forename: Alessio
authors.initials: A
authors.lastname: Cappetti
authors.forename: Claudio
authors.initials: C
authors.lastname: Feliciani
authors.forename: Paolo
authors.initials: P
authors.lastname: Fabbri
mesh_terms: D000051:Acantholysis / Q000276:immunology / Q000473:pathology; D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D001706:Biopsy; D019013:CD40 Antigens / Q000032:analysis*; D023201:CD40 Ligand / Q000032:analysis*; D051183:Desmoglein 1 / Q000276:immunology; D051184:Desmoglein 3 / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000097:blood / Q000276:immunology* / Q000473:pathology*; D012333:RNA, Messenger / Q000032:analysis / Q000302:isolation & purification; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012867:Skin / Q000473:pathology
chemical_list: D001323:Autoantibodies; D019013:CD40 Antigens; D051183:Desmoglein 1; D051184:Desmoglein 3; D012333:RNA, Messenger; D023201:CD40 Ligand