Transition from Bullous Pemphigoid to Pemphigus Foliaceus: intermolecular epitope spreading thirteen years after initial diagnosis

Abstract: Bullous pemphigoid (BP) is a prototypic autoimmune blistering disease, characterized by tissue-bound and circulating autoantibodies directed against collagen XVII/ BP180 and/or dystonin/BP230, both components of the hemidesmosomes involved in dermal–epidermal cohesion (1). Epitope spreading (ES) represents the process of diversification of B-cell and/or T-cell response from the initial dominant epitope to a secondary epitope over time. ES can occur within a single antigen (intramolecular) or involve different antigens (intermolecular) and manifest at various stages of the disease (2, 3). Some ES events appear to be quite common, e.g. shifting between pemphigus vulgaris and pemphigus foliaceus (PF), whereas others are rare. ES, for example, has also been shown in 17 of 35 patients with BP in a multicentre prospective study with a 12-month observation period. The ES occurred preferentially at an early stage of the disease, shaping the course of BP disease (4). However, ES phenomena may also lead to disease transition. We report here a patient with long-standing BP in clinical, but not serological remission, who developed typical PF 13 years after diagnosis of BP. Although a rare event of second autoimmunity cannot be excluded, the serological IgG subclass shift, with transient presentation of features of both BP and PF, is more indicative for epitope spreading

Object Details

• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch
• Notes: Acta dermato venereologica. - 99, 11 (2019) , 1029-1030, ISSN: 1651-2057

Contributors, Places and Time

• Event: Veröffentlichung
• (where): Freiburg
• (who): Universität
• (when): 2019
• Creator: Schauer, Franziska ; Steinke, Holger ; Thoma, Käthe ; Kiritsi, Dimitra

Further information

• DOI: 10.2340/00015555-3250
• URN: urn:nbn:de:bsz:25-freidok-1511027
• Rights: Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 25.03.2025, 1:43 PM CET