Desmoglein 1 and 3 enzyme-linked immunosorbent assay in Iranian patients with pemphigus vulgaris: correlation with phenotype, severity, and disease activity

• Published in: Journal of the European Academy of Dermatology and Venereology 2007-11, Vol.21 (10), p.1319-1324
• Main Authors: Daneshpazhooh, M, Chams-Davatchi, C, Khamesipour, A, Mansoori, P, Taheri, A, Firooz, A, Mortazavi, H, Esmaili, N, Dowlati, Y
• Format: Article
• Language: English
• Subjects: activity; Adolescent; Adult; Aged; Aged, 80 and over; Analysis of Variance; Autoantibodies; Biomarkers - metabolism; Child; desmoglein; desmoglein 1; Desmoglein 1 - immunology; Desmoglein 1 - metabolism; desmoglein 3; Desmoglein 3 - immunology; Desmoglein 3 - metabolism; Enzyme-linked immunosorbent assay; Enzyme-Linked Immunosorbent Assay - methods; Female; Hospitals; Humans; Immunoglobulin G; Iran; Male; Middle Aged; Mucosa; Pemphigus; Pemphigus - diagnosis; Pemphigus - immunology; Pemphigus - pathology; pemphigus vulgaris; Phenotype; Predictive Value of Tests; Remission; Sensitivity and Specificity; severity; Severity of Illness Index; Skin diseases; Statistics, Nonparametric
• ISSN: 0926-9959; 1468-3083
• DOI: 10.1111/j.1468-3083.2007.02254.x

Background  Pemphigus vulgaris (PV) is a chronic autoimmune blistering disorder of the skin and mucosa characterized by the presence of autoantibodies against desmoglein3 (Dsg3). Some patients also have antibodies against desmoglein1 (Dsg1). The aims of this study were to evaluate the diagnostic value of Dsg enzyme‐linked immunosorbent assay (ELISA) in Iranian PV patients, to assess its correlation with the clinical phenotype and severity of disease and to investigate the changes of these antibodies after treatment. Methods  Seventy‐three patients with PV (29 men, 44 women) presenting to the Pemphigus Research Unit at Razi Hospital, Tehran, Iran were enrolled. ELISAs were used to detect IgG autoantibodies reactive with the ectodomains of Dsg1 and Dsg3, and the correlation of antibodies with the clinical phenotype as well as oral and skin disease severity was assessed. In addition, the tests were repeated in 18 patients after treatment and the resulting remission. Results  Anti‐Dsg1 and anti‐Dsg3 were detected in 56 (76.7%) and 69 (94.5%) patients, respectively. Anti‐Dsg1 and anti‐Dsg3 antibodies were present in 48 (94.1%) and 50 (98%) patients with mucocutaneous type, in 2 (12.5%) and 15 (93.7%) patients with mucosal type, and in 6 (100%) and 4 (66.7%) patients with cutaneous PV, respectively. The mean anti‐Dsg1 index values were significantly higher in cutaneous and mucocutaneous phenotypes than mucosal PV (P