{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Rastegar Lari T"],"funding":["CRC 1526 Pathomechanisms of Antibody-mediated Autoimmunity","University of Lübeck","Schleswig-Holstein Excellence Cluster Precision Medicine in Chronic Inflammation","CRU 303 Pemphigoid Diseases","DFG - Deutsche Forschungsgemeinschaft"],"pagination":["218"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC11857079"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["15(2)"],"pubmed_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, <i>n</i> = 65; BP-cohort 2, <i>n</i> = 119), pemphigus vulgaris (PV, <i>n</i> = 67), and pemphigus foliaceus (PF, <i>n</i> = 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."],"journal":["Life (Basel, Switzerland)"],"pubmed_title":["Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression."],"pmcid":["PMC11857079"],"funding_grant_id":["DFG EM 362/1-1","DFG EXC 2167/1, TI-3","J13-2022"],"pubmed_authors":["Macias L","Prußmann J","Robrahn L","Schmidt E","Dikmen HO","Hoffmann MH","Rastegar Lari T","Emtenani S","Kiehne C","Szymczak S","Engster S","van Beek N","Weyers I"],"additional_accession":[]},"is_claimable":false,"name":"Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression.","description":"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, <i>n</i> = 65; BP-cohort 2, <i>n</i> = 119), pemphigus vulgaris (PV, <i>n</i> = 67), and pemphigus foliaceus (PF, <i>n</i> = 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.","dates":{"release":"2025-01-01T00:00:00Z","publication":"2025 Jan","modification":"2025-04-22T18:57:41.852Z","creation":"2025-04-06T02:37:04.901Z"},"accession":"S-EPMC11857079","cross_references":{"pubmed":["40003627"],"doi":["10.3390/life15020218"]}}