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Life
Volume 15
Issue 2
10.3390/life15020218
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This is an early access version, the complete PDF, HTML, and XML versions will be available soon.
Article

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

by
Tina Rastegar Lari
1,
Louis Macias
2,
Lara Robrahn
3,4,
Hasan Onur Dikmen
1,3,
Jasper Prüßmann
3,
Charlotte Kiehne
1,3,
Simon Engster
1,
Imke Weyers
5,
Silke Szymczak
2,
Nina van Beek
3,
Markus H. Hoffmann
3,4,
Enno Schmidt
1,3 and
Shirin Emtenani
1,*
1
Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck 23562, Germany
2
Institute of Medical Biometry and Statistics, University of Lübeck, Lübeck 23562, Germany
3
Department of Dermatology, Allergology and Venerology, University of Lübeck, Lübeck 23562, Germany
4
Institute for Systemic Inflammation Research, University of Lübeck, Lübeck 23562, Germany
5
Institute of Anatomy, University of Lübeck, Lübeck 23562, Germany
*
Author to whom correspondence should be addressed.
Life 2025, 15(2), 218; https://doi.org/10.3390/life15020218 (registering DOI)
Submission received: 10 December 2024 / Revised: 22 January 2025 / Accepted: 30 January 2025 / Published: 31 January 2025
(This article belongs to the Section Medical Research)
Versions Notes

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.
Keywords: autoimmune blistering disease; bullous pemphigoid; pemphigus; predilection sites; antigen expression autoimmune blistering disease; bullous pemphigoid; pemphigus; predilection sites; antigen expression

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MDPI and ACS Style

Rastegar Lari, T.; Macias, L.; Robrahn, L.; Dikmen, H.O.; Prüßmann, J.; Kiehne, C.; Engster, S.; Weyers, I.; Szymczak, S.; van Beek, N.; et al. Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression. Life 2025, 15, 218. https://doi.org/10.3390/life15020218

AMA Style

Rastegar Lari T, Macias L, Robrahn L, Dikmen HO, Prüßmann J, Kiehne C, Engster S, Weyers I, Szymczak S, van Beek N, et al. Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression. Life. 2025; 15(2):218. https://doi.org/10.3390/life15020218

Chicago/Turabian Style

Rastegar Lari, Tina, Louis Macias, Lara Robrahn, Hasan Onur Dikmen, Jasper Prüßmann, Charlotte Kiehne, Simon Engster, Imke Weyers, Silke Szymczak, Nina van Beek, and et al. 2025. "Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression" Life 15, no. 2: 218. https://doi.org/10.3390/life15020218

APA Style

Rastegar Lari, T., Macias, L., Robrahn, L., Dikmen, H. O., Prüßmann, J., Kiehne, C., Engster, S., Weyers, I., Szymczak, S., van Beek, N., Hoffmann, M. H., Schmidt, E., & Emtenani, S. (2025). Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression. Life, 15(2), 218. https://doi.org/10.3390/life15020218

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