title: Late development of antidesmoglein 1 antibodies in pemphigus vulgaris: correlation with disease progression.
abstract: The coexistence of antidesmoglein 3 (Dsg3) and antidesmoglein 1 (Dsg1) autoantibodies is well described in patients with pemphigus vulgaris (PV); however, there is little evidence of sequential development of these two autoantibodies. Autoantibody responses to Dsg3 and Dsg1 were studied in seven PV patients over time by enzyme-linked immunosorbent assay, using baculovirus expressed recombinant fusion proteins. All patients had anti-Dsg3 IgG antibodies at presentation. Two patients developed anti-Dsg1 later in the course of the disease. The transition in autoantibody profile was associated with disease progression to generalized PV involving mucous membranes and skin in both patients; one patient initially presented with a predominantly mucosal phenotype, the other with herpetiform pemphigus-like features. These findings demonstrate that there is an extension of autoimmune response from anti-Dsg3 only to both anti-Dsg3 and anti-Dsg1 in some patients, which is associated with an alteration in clinical expression in PV.
authors.affiliation: Department of Dermatology, Nara Medical University, Kashihara City, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Miyagawa
authors.forename: M
authors.initials: M
authors.lastname: Amagai
authors.forename: T
authors.initials: T
authors.lastname: Iida
authors.forename: Y
authors.initials: Y
authors.lastname: Yamamoto
authors.forename: T
authors.initials: T
authors.lastname: Nishikawa
authors.forename: T
authors.initials: T
authors.lastname: Shirai
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000378:metabolism*; D001327:Autoimmune Diseases / Q000276:immunology*; D003598:Cytoskeletal Proteins / Q000276:immunology*; D051183:Desmoglein 1; D051180:Desmoplakins; D003896:Desmosomes / Q000276:immunology*; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology*; D013997:Time Factors
chemical_list: D001323:Autoantibodies; D003598:Cytoskeletal Proteins; C495297:DSG1 protein, human; D051183:Desmoglein 1; D051180:Desmoplakins