Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression

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Abstract
Autoimmune blistering diseases (AIBDs) involve autoantibodies targeting proteins in the epidermal/epithelial desmosome (pemphigus) or basement membrane zone (pemphigoid). Despite widespread antigen distribution, lesions exhibit a scattered involvement pattern. This study maps the frequency/severity of AIBD lesions on various body parts and investigates whether differential antigen expression contributes to specific predilection sites. We analyzed affected sites presenting blisters/erosions, erythematous/urticarial lesions, and mucosal lesions in bullous pemphigoid (BP-cohort 1, n = 65; BP-cohort 2, n = 119), pemphigus vulgaris (PV, n = 67), and pemphigus foliaceus (PF, n = 20) patients. To assess antigen expression, we conducted indirect immunofluorescence (IF) staining of 11 AIBD antigens from 13 anatomical sites of 10 body donors without AIBD. In BP, blisters/erosions and erythematous/urticarial lesions predominantly affected arms and legs, while PV/PF patients exhibited frequent involvement of buccal mucosa and back, respectively. IF staining identified significant regional differences in BP180, BP230, and integrin β4 expression, although these variations did not correlate with a higher lesion frequency/severity. Other antigens showed consistent expression across all regions. Our findings suggest that predilection sites for BP and PV/PF are largely unaffected by regional variations in antigen expression but may be influenced by factors like microbiota, mechanical stress, sunlight exposure, local immunity, or genetics.
Funding Information
  • German Research Foundation (DFG EXC 2167/1, TI-3, J13-2022, DFG EM 362/1-1)
  • CRU 303 Pemphigoid Diseases (DFG EXC 2167/1, TI-3, J13-2022, DFG EM 362/1-1)
  • CRC 1526 Pathomechanisms of Antibody-mediated Autoimmunity (DFG EXC 2167/1, TI-3, J13-2022, DFG EM 362/1-1)
  • Universität zu Lübeck (DFG EXC 2167/1, TI-3, J13-2022, DFG EM 362/1-1)
  • DFG-deutsche forschungsgemeinschaft (DFG EXC 2167/1, TI-3, J13-2022, DFG EM 362/1-1)
  • RTG2633 Defining and Targeting Autoimmune Pre-Disease (DFG EXC 2167/1, TI-3, J13-2022, DFG EM 362/1-1)

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