title: Clinical activity of pemphigus vulgaris relates to IgE autoantibodies against desmoglein 3.
abstract: Pemphigus vulgaris (PV) is a severe autoimmune bullous skin disease and is primarily associated with IgG against desmoglein 3 (dsg3), a desmosomal adhesion protein. In light of the recent association of autoreactive T helper (Th) 2 cells with active PV, the present study sought to relate the occurrence of Th2-regulated dsg3-specific autoantibody subtypes, i.e. IgE and IgG4, in 93 well-characterized PV patients. Patients with acute onset PV (n=37) showed the highest concentrations of serum IgE and IgG4 autoantibodies, which were significantly lower in PV patients in remission (n=14). Furthermore, there was a strong correlation between dsg3-reactive IgE and IgG4 in acute onset, but not in chronic active (n=42) or remittent patients. Additionally, intercellular IgE deposits were detected in the epidermis of acute onset PV. Thus, dsg3-specific IgE and IgG4 autoantibodies are related to acute onset disease which provides additional support to the concept that PV is a Th2-driven autoimmune disorder.
authors.affiliation: Department of Dermatology and Allergology, Philipps University Marburg, Germany. nagela@med.uni-marburg.de
authors.forename: Angela
authors.initials: A
authors.lastname: Nagel
authors.forename: Angelika
authors.initials: A
authors.lastname: Lang
authors.forename: Dorothee
authors.initials: D
authors.lastname: Engel
authors.forename: Eva
authors.initials: E
authors.lastname: Podstawa
authors.forename: Nicolas
authors.initials: N
authors.lastname: Hunzelmann
authors.forename: Ornella
authors.initials: O
authors.lastname: de Pita
authors.forename: Luca
authors.initials: L
authors.lastname: Borradori
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Uter
authors.forename: Michael
authors.initials: M
authors.lastname: Hertl
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D051184:Desmoglein 3 / Q000276:immunology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007073:Immunoglobulin E / Q000276:immunology*; D007074:Immunoglobulin G / Q000276:immunology; D007223:Infant; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology*; D012372:ROC Curve; D011994:Recombinant Proteins / Q000276:immunology; D012737:Sex Factors; D018418:Th2 Cells / Q000276:immunology; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D051184:Desmoglein 3; D007074:Immunoglobulin G; D011994:Recombinant Proteins; D007073:Immunoglobulin E