Clinical and immunoserological characteristics of anti‐p200 pemphigoid: Comparisons of patients with epitope spreading and non‐epitope spreading

Abstract

BackgroundAnti‐p200 pemphigoid is a rare autoimmune subepidermal blistering disease. Although the phenomenon of epitope spreading has been reported to be common in anti‐p200 pemphigoid, the association between its clinical and immunoserological features has yet to be elucidated.ObjectivesOur aim was to compare the clinical and immunoserological characteristics of anti‐p200 pemphigoid patients with and without epitope spreading.MethodsWe performed a retrospective cohort study encompassing 30 patients with anti‐p200 pemphigoid between January 2015 and December 2022. The clinical and immunoserological characteristics of anti‐p200 pemphigoid were analyzed using combined immunoserological assays.ResultsEpitope spreading was observed in 11 of 30 patients (36.7%) with anti‐p200 pemphigoid. Compared with patients in the non‐epitope spreading group, patients in the epitope spreading group showed more heterogeneous clinical presentations (P = 0.018), a higher proportion of mucosal involvement (P = 0.003), higher Bullous Pemphigoid Disease Area Index (BPDAI) scores for skin erosions/blisters (P = 0.018), mucosal erosions/blisters (P = 0.001), activity (P = 0.017) and total scores (P = 0.022), and required a higher initial dose of prednisone for disease control (P = 0.040).ConclusionsThis study supported the idea that anti‐p200 pemphigoid was prone to epitope spreading. Anti‐p200 pemphigoid patients with epitope spreading are more likely to present heterogeneous clinical phenotypes, frequent mucosal involvement, and a more severe and recalcitrant disease course.