{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T20:49:28Z","timestamp":1790887768849,"version":"4.1.0"},"reference-count":92,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2022,12,6]],"date-time":"2022-12-06T00:00:00Z","timestamp":1670284800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Lichen sclerosus (LS) is an acquired chronic inflammatory dermatosis predominantly affecting the anogenital area with recalcitrant itching and soreness. Progressive or persistent LS may cause urinary and sexual disturbances and an increased risk of local skin malignancy with a prevalence of up to 11%. Investigations on lipoid proteinosis, an autosomal recessive genodermatosis caused by loss-of-function mutations in the extracellular matrix protein 1 (ECM1) gene, led to the discovery of a humoral autoimmune response to the identical molecule in LS, providing evidence for an autoimmune and genetic counterpart targeting ECM1. This paper provides an overview of the fundamental importance and current issue of better understanding the immunopathology attributed to ECM1 in LS. Furthermore, we highlight the pleiotropic action of ECM1 in homeostatic and structural maintenance of skin biology as well as in a variety of human disorders possibly associated with impaired or gained ECM1 function, including the inflammatory bowel disease ulcerative colitis, Th2 cell-dependent airway allergies, T-cell and B-cell activation, and the demyelinating central nervous system disease multiple sclerosis, to facilitate sharing the concept as a plausible therapeutic target of this attractive molecule.<\/jats:p>","DOI":"10.3390\/diagnostics12123070","type":"journal-article","created":{"date-parts":[[2022,12,7]],"date-time":"2022-12-07T02:49:35Z","timestamp":1670381375000},"page":"3070","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":27,"title":["Lichen Sclerosus: A Current Landscape of Autoimmune and Genetic Interplay"],"prefix":"10.3390","volume":"12","author":[{"given":"Noritaka","family":"Oyama","sequence":"first","affiliation":[{"name":"Department of Dermatology, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuoka-Shimoaizuki, Eiheiji, Fukui 910-1193, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3738-2682","authenticated-orcid":false,"given":"Minoru","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Faculty of Medical Sciences, University of Fukui, 23-3 Matsuoka-Shimoaizuki, Eiheiji, Fukui 910-1193, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,6]]},"reference":[{"key":"ref_1","first-page":"477","article-title":"Prevalence of Vulvar Lichen Sclerosus in a General Gynecology Practice","volume":"50","author":"Goldstein","year":"2005","journal-title":"J. Reprod. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1111\/j.1468-3083.2011.04155.x","article-title":"Clinical Parameters in Male Genital Lichen Sclerosus: A Case Series of 329 Patients","volume":"26","author":"Edmonds","year":"2012","journal-title":"J. Eur. Acad. Dermatology Venereol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"86","DOI":"10.1007\/s004040100209","article-title":"Survey of Genital Lichen Sclerosus in Women and Men","volume":"266","author":"Hagedorn","year":"2002","journal-title":"Arch. Gynecol. Obstet."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1111\/jdv.13136","article-title":"Evidence-Based (S3) Guideline on (Anogenital) Lichen Sclerosus","volume":"29","author":"Kirtschig","year":"2015","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1224","DOI":"10.1158\/1055-9965.EPI-16-0019","article-title":"Lichen Sclerosus: Incidence and Risk of Vulvar Squamous Cell Carcinoma","volume":"25","author":"Bleeker","year":"2016","journal-title":"Cancer Epidemiol. Biomark. Prev."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1097\/PGP.0000000000000341","article-title":"The Prevalence of Lichen Sclerosus in Patients with Vulvar Squamous Cell Carcinoma","volume":"36","author":"Davick","year":"2017","journal-title":"Int. J. Gynecol. Pathol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1097\/LGT.0000000000000186","article-title":"Vulvar Lichen Sclerosus and Neoplastic Transformation: A Retrospective Study of 976 Cases","volume":"20","author":"Micheletti","year":"2016","journal-title":"J. Low. Genit. Tract. Dis."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.jogc.2021.09.023","article-title":"Risk of Vulvar Squamous Cell Carcinoma in Lichen Sclerosus and Lichen Planus: A Systematic Review","volume":"44","author":"Leis","year":"2022","journal-title":"J. Obstet. Gynaecol. Canada"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1111\/1346-8138.14171","article-title":"Diagnostic Criteria, Severity Classification and Guidelines of Lichen Sclerosus et Atrophicus","volume":"45","author":"Hasegawa","year":"2018","journal-title":"J. Dermatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1111\/j.1365-2133.2010.09997.x","article-title":"British Association of Dermatologists\u2019 Guidelines for the Management of Lichen Sclerosus 2010","volume":"163","author":"Neill","year":"2010","journal-title":"Br. J. Dermatol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"84","DOI":"10.1016\/j.jaad.2014.02.019","article-title":"A Double-Blind, Randomized Prospective Study Evaluating Topical Clobetasol Propionate 0.05% versus Topical Tacrolimus 0.1% in Patients with Vulvar Lichen Sclerosus","volume":"71","author":"Funaro","year":"2014","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1016\/j.jaad.2007.03.006","article-title":"Open-Label Trial of Cyclosporine for Vulvar Lichen Sclerosus","volume":"57","author":"Turan","year":"2007","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_13","first-page":"614","article-title":"Open Study of Topical 0.025% Tretinoin in the Treatment of Vulvar Lichen Sclerosus: One Year of Therapy","volume":"40","author":"Virgili","year":"1995","journal-title":"J. Reprod. Med. Obstet. Gynecol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1001\/jamadermatol.2013.7733","article-title":"UV-A1 Phototherapy vs Clobetasol Propionate, 0.05%, in the Treatment of Vulvar Lichen Sclerosus: A Randomized Clinical Trial","volume":"150","author":"Terras","year":"2014","journal-title":"JAMA Dermatol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/j.ejogrb.2008.07.027","article-title":"An Open Trial of 5-Aminolevulinic Acid Photodynamic Therapy for Vulvar Lichen Sclerosus","volume":"141","author":"Sotiriou","year":"2008","journal-title":"Eur. J. Obstet. Gynecol. Reprod. Biol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1111\/j.1365-2133.1986.tb02831.x","article-title":"The Development of Lichen Sclerosus et Atrophicus in Monozygotic Twin Girls","volume":"114","author":"Thomas","year":"1986","journal-title":"Br. J. Dermatol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1111\/j.1525-1470.1994.tb00572.x","article-title":"Familial Lichen Sclerosus et Atrophicus in Childhood","volume":"11","author":"Sahn","year":"1994","journal-title":"Pediatr. Dermatol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1046\/j.1365-2133.1999.02611.x","article-title":"Lichen Sclerosus in Adult Men: A Study of HLA Associations and Susceptibility to Autoimmune Disease","volume":"140","author":"Azurdia","year":"1999","journal-title":"Br. J. Dermatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1111\/j.1365-2133.1995.tb05013.x","article-title":"The Association between Lichen Sclerosus and Antigens of the HLA System","volume":"132","author":"Marren","year":"1995","journal-title":"Br. J. Dermatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1111\/j.0022-202X.2005.23905.x","article-title":"The Association between HLA DR, DQ Antigens, and Vulval Lichen Sclerosus in the UK: HLA DRB112 and Its Associated DRB112\/DQB10301\/04\/09\/010 Haplotype Confers Susceptibility to Vulval Lichen Sclerosus, and HLA DRB10301\/04 and Its Associated DRB10301\/04\/DQB10201\/02\/03 Haplotype Protects from Vulval Lichen Sclerosus","volume":"125","author":"Gao","year":"2005","journal-title":"J. Investig. Dermatol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1007\/978-3-030-92616-8_7","article-title":"The Immunogenetics of Morphea and Lichen Sclerosus","volume":"1367","year":"2022","journal-title":"Adv. Exp. Med. Biol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"146","DOI":"10.3389\/fendo.2017.00146","article-title":"Thyroid Autoimmunity and Lichen","volume":"8","author":"Guarneri","year":"2017","journal-title":"Front. Endocrinol."},{"key":"ref_23","first-page":"482","article-title":"Lichen Sclerosus et Atrophicus. A Study of 76 Cases and Their Relation to Diabetes","volume":"19","author":"Camacho","year":"1988","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1111\/j.1365-2133.1981.tb08172.x","article-title":"An Investigation into the Incidence of Auto-immune Disorders in Patients with Lichen Sclerosus and Atrophicus","volume":"104","author":"Harrington","year":"1981","journal-title":"Br. J. Dermatol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1111\/j.1365-2133.1988.tb01748.x","article-title":"Lichen Sclerosus et Atrophicus and Autoimmunity\u2014a Study of 350 Women","volume":"118","author":"Thomas","year":"1988","journal-title":"Br. J. Dermatol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1111\/j.1468-3083.2009.03375.x","article-title":"T Cells Reactive with the NC16A Domain of BP180 Are Present in Vulval Lichen Sclerosus and Lichen Planus","volume":"24","author":"Baldo","year":"2010","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1097\/LGT.0b013e31825e9b18","article-title":"Lichen Sclerosus and Immunobullous Disease","volume":"16","author":"Walsh","year":"2012","journal-title":"J. Low. Genit. Tract Dis."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1093\/hmg\/11.7.833","article-title":"Lipoid Proteinosis Maps to 1q21 and Is Caused by Mutations in the Extracellular Matrix Protein 1 Gene (ECM1)","volume":"11","author":"Hamada","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/S0140-6736(03)13863-9","article-title":"Autoantibodies to Extracellular Matrix Protein 1 in Lichen Sclerosus","volume":"362","author":"Oyama","year":"2003","journal-title":"Lancet"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1550","DOI":"10.1172\/JCI200420373","article-title":"Development of Antigen-Specific ELISA for Circulating Autoantibodies to Extracellular Matrix Protein 1 in Lichen Sclerosus","volume":"113","author":"Oyama","year":"2004","journal-title":"J. Clin. Investig."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1038\/ni.1983","article-title":"ECM1 Controls T(H)2 Cell Egress from Lymph Nodes through Re-Expression of S1P(1)","volume":"12","author":"Li","year":"2011","journal-title":"Nat. Immunol."},{"key":"ref_32","first-page":"S70","article-title":"Zebrafish Model of Lipoid Proteinosis","volume":"131","author":"Li","year":"2011","journal-title":"J. Investig. Dermatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"8621","DOI":"10.1073\/pnas.1801196115","article-title":"Extracellular Matrix Protein 1 Promotes Follicular Helper T Cell Differentiation and Antibody Production","volume":"115","author":"He","year":"2018","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1038\/ng.145","article-title":"Genetic Determinants of Ulcerative Colitis Include the ECM1 Locus and Five Loci Implicated in Crohn\u2019s Disease","volume":"40","author":"Fisher","year":"2008","journal-title":"Nat. Genet."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"3083","DOI":"10.1073\/pnas.1912774117","article-title":"ECM1 Is an Essential Factor for the Determination of M1 Macrophage Polarization in IBD in Response to LPS Stimulation","volume":"117","author":"Zhang","year":"2020","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1197","DOI":"10.1093\/intimm\/dxl060","article-title":"Foxp3-Dependent and -Independent Molecules Specific for CD25+CD4+ Natural Regulatory T Cells Revealed by DNA Microarray Analysis","volume":"18","author":"Sugimoto","year":"2006","journal-title":"Int. Immunol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1111\/j.1464-410X.2011.10368.x","article-title":"Development of Male Genital Lichen Sclerosus in Penile Reconstruction Skin Grafts after Cancer Surgery: An Unreported Complication","volume":"109","author":"Abdelbaky","year":"2012","journal-title":"BJU Int."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1159\/000356163","article-title":"Prospective Clinical and Epidemiologic Study of Vulvar Lichen Sclerosus: Analysis of Prevalence and Severity of Clinical Features, Together with Historical and Demographic Associations","volume":"228","author":"Virgili","year":"2014","journal-title":"Dermatology"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"1458","DOI":"10.1001\/archdermatol.2009.313","article-title":"A Case of Lichen Sclerosus of the Scalp Associated with Autoantibodies to Extracellular Matrix Protein 1","volume":"145","author":"Kawakami","year":"2009","journal-title":"Arch. Dermatol."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"e919353-1","DOI":"10.12659\/AJCR.919353","article-title":"Hemorrhagic Bullous Lichen Sclerosus: A Case Report","volume":"21","author":"Khatib","year":"2020","journal-title":"Am. J. Case Rep."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1111\/bjd.12014","article-title":"Peristomal Lichen Sclerosus: The Role of Occlusion and Urine Exposure?","volume":"168","author":"Lyon","year":"2013","journal-title":"Br. J. Dermatol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1111\/bjd.12137","article-title":"Quality of Life in Dutch Women with Lichen Sclerosus","volume":"168","author":"Lansdorp","year":"2013","journal-title":"Br. J. Dermatol."},{"key":"ref_43","first-page":"9","article-title":"Lichen Sclerosus et Atrophicus","volume":"57","author":"Wallace","year":"1971","journal-title":"Trans. St. Johns. Hosp. Dermatol. Soc."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1067\/mjd.2001.113474","article-title":"Childhood Vulvar Lichen Sclerosus: An Increasingly Common Problem","volume":"44","author":"Powell","year":"2001","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"522","DOI":"10.1016\/j.juro.2010.09.107","article-title":"Lichen Sclerosus: Epidemiological Distribution in an Equal Access Health Care System","volume":"185","author":"Nelson","year":"2011","journal-title":"J. Urol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1097\/00007611-200301000-00004","article-title":"Balanitis Xerotica Obliterans: Epidemiologic Distribution in an Equal Access Health Care System","volume":"96","author":"Kizer","year":"2003","journal-title":"South. Med. J."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1111\/cup.12032","article-title":"Dermatopathology of the Foreskin: An Institutional Experience of over 400 Cases","volume":"40","author":"West","year":"2013","journal-title":"J. Cutan. Pathol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0167-4943(00)00059-5","article-title":"Vulvovaginal Examinations in Elderly Nursing Home Women Residents","volume":"31","author":"Leibovitz","year":"2000","journal-title":"Arch. Gerontol. Geriatr."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1097\/LGT.0000000000000669","article-title":"The Impact of Psychosexual Counseling in Women With Lichen Sclerosus: A Randomized Controlled Trial","volume":"26","author":"Vittrup","year":"2022","journal-title":"J. Low. Genit. Tract Dis."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1089\/jwh.2014.4805","article-title":"The Impact of Vulvar Lichen Sclerosus on Sexual Dysfunction","volume":"23","author":"Haefner","year":"2014","journal-title":"J. Women\u2019s Health"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.1002\/ijc.30621","article-title":"Lichen Sclerosus and Risk of Cancer","volume":"140","author":"Halonen","year":"2017","journal-title":"Int. J. Cancer"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1001\/jamadermatol.2015.0643","article-title":"Long-Term Management of Adult Vulvar Lichen Sclerosus: A Prospective Cohort Study of 507 Women","volume":"151","author":"Lee","year":"2015","journal-title":"JAMA Dermatol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1111\/j.1365-2559.2005.02209.x","article-title":"Early Vulvar Lichen Sclerosus: A Histopathological Challenge","volume":"47","author":"Regauer","year":"2005","journal-title":"Histopathology"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1097\/PGP.0b013e3181a9fb0d","article-title":"Diagnosis of Vulval Inflammatory Dermatoses: A Pathological Study with Clinical Correlation","volume":"28","author":"Niamh","year":"2009","journal-title":"Int. J. Gynecol. Pathol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1006\/geno.1997.4918","article-title":"The Human Extracellular Matrix Gene 1 (ECM1): Genomic Structure, CDNA Cloning, Expression Pattern, and Chromosomal Localization","volume":"45","author":"Smits","year":"1997","journal-title":"Genomics"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/S0945-053X(97)90017-2","article-title":"Characterization of the Human Extracellular Matrix Protein 1 Gene on Chromosome 1q21","volume":"16","author":"Johnson","year":"1997","journal-title":"Matrix Biol."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"903","DOI":"10.1002\/jbmr.5650090616","article-title":"Characterization of the Osteogenic Stromal Cell Line MN7: Identification of Secreted MN7 Proteins Using Two-Dimensional Polyacrylamide Gel Electrophoresis, Western Blotting, and Microsequencing","volume":"9","author":"Mathieu","year":"1994","journal-title":"J. Bone Miner. Res."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/S0378-1119(98)00558-7","article-title":"Molecular Cloning and Characterization of the Mouse Ecm1 Gene and Its 5\u2019 Regulatory Sequences","volume":"226","author":"Smits","year":"1999","journal-title":"Gene"},{"key":"ref_59","first-page":"44","article-title":"Importance of Extracellular Matrix Protein 1 (ECM1) in Maintaining the Functional Integrity of the Human Skin","volume":"3","author":"Sercu","year":"2009","journal-title":"Undefined"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1111\/j.1365-2133.2005.07001.x","article-title":"Expression of Extracellular Matrix Protein 1 (ECM1) in Human Skin Is Decreased by Age and Increased upon Ultraviolet Exposure","volume":"154","author":"Sander","year":"2006","journal-title":"Br. J. Dermatol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"17491","DOI":"10.1074\/jbc.M210529200","article-title":"Perlecan Protein Core Interacts with Extracellular Matrix Protein 1 (ECM1), a Glycoprotein Involved in Bone Formation and Angiogenesis","volume":"278","author":"Mongiat","year":"2003","journal-title":"J. Biol. Chem."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1111\/j.1600-0625.2005.00374.x","article-title":"A Novel Splice-Site Mutation in ECM-1 Gene in a Consanguineous Family with Lipoid Proteinosis","volume":"14","author":"Horev","year":"2005","journal-title":"Exp. Dermatol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1046\/j.1523-1747.2000.00916.x","article-title":"Differentiation-Dependent Alternative Splicing and Expression of the Extracellular Matrix Protein 1 Gene in Human Keratinocytes","volume":"114","author":"Smits","year":"2000","journal-title":"J. Investig. Dermatol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"1397","DOI":"10.1038\/sj.jid.5701231","article-title":"Interaction of Extracellular Matrix Protein 1 with Extracellular Matrix Components: ECM1 Is a Basement Membrane Protein of the Skin","volume":"128","author":"Sercu","year":"2008","journal-title":"J. Investig. Dermatol."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"276","DOI":"10.1016\/j.matbio.2010.01.007","article-title":"Interaction between Cartilage Oligomeric Matrix Protein and Extracellular Matrix Protein 1 Mediates Endochondral Bone Growth","volume":"29","author":"Kong","year":"2010","journal-title":"Matrix Biol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"37823","DOI":"10.1074\/jbc.M110.136408","article-title":"Phospholipid Scramblase 1 Is Secreted by a Lipid Raft-Dependent Pathway and Interacts with the Extracellular Matrix Protein 1 in the Dermal Epidermal Junction Zone of Human Skin","volume":"285","author":"Merregaert","year":"2010","journal-title":"J. Biol. Chem."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.matbio.2009.02.003","article-title":"ECM1 Interacts with Fibulin-3 and the Beta 3 Chain of Laminin 332 through Its Serum Albumin Subdomain-like 2 Domain","volume":"28","author":"Sercu","year":"2009","journal-title":"Matrix Biol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1016\/j.bbrc.2005.06.046","article-title":"Extracellular Matrix Protein 1 Interacts with the Domain III of Fibulin-1C and 1D Variants through Its Central Tandem Repeat 2","volume":"333","author":"Fujimoto","year":"2005","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/j.jdermsci.2020.06.010","article-title":"Gene Silencing of Extracellular Matrix Protein 1 (ECM1) Results in Phenotypic Alterations of Dermal Fibroblasts Reminiscent of Clinical Features of Lichen Sclerosus","volume":"100","author":"Utsunomiya","year":"2020","journal-title":"J. Dermatol. Sci."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"988","DOI":"10.1096\/fsb2fj990934com","article-title":"Extracellular Matrix Protein 1 (ECM1) Has Angiogenic Properties and Is Expressed by Breast Tumor Cells","volume":"15","author":"Han","year":"2001","journal-title":"FASEB J."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1016\/S8756-3282(00)00428-2","article-title":"Recombinant Human Extracellular Matrix Protein 1 Inhibits Alkaline Phosphatase Activity and Mineralization of Mouse Embryonic Metatarsals in Vitro","volume":"28","author":"Deckers","year":"2001","journal-title":"Bone"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1080\/07357900701788148","article-title":"The Extracellular Matrix Protein 1: Its Molecular Interaction and Implication in Tumor Progression","volume":"26","author":"Sercu","year":"2008","journal-title":"Cancer Investig."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1031","DOI":"10.1111\/j.1468-3083.2010.03572.x","article-title":"The High Rate of Familial Lichen Sclerosus Suggests a Genetic Contribution: An Observational Cohort Study","volume":"24","author":"Sherman","year":"2010","journal-title":"J. Eur. Acad. Dermatol. Venereol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1136\/bmj.4.5936.78","article-title":"Organ-Specific Antibodies in Patients with Lichen Sclerosus","volume":"4","author":"Goolamali","year":"1974","journal-title":"Br. Med. J."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"396","DOI":"10.1001\/jama.1913.04350060018011","article-title":"Idiopathic Atrophy of the Skin","volume":"61","author":"Irvine","year":"1913","journal-title":"JAMA"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1111\/j.1365-2230.2004.01440.x","article-title":"The Role of Extracellular Matrix Protein 1 in Human Skin","volume":"29","author":"Chan","year":"2004","journal-title":"Clin. Exp. Dermatol."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"157","DOI":"10.2340\/0001555561157161","article-title":"An Appraisal of Routine Direct Immunofluorescence in Vulvar Disorders","volume":"61","author":"Bushkell","year":"1981","journal-title":"Acta Derm. Venereol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1111\/j.1600-0625.2007.00608.x","article-title":"The Molecular Basis of Lipoid Proteinosis: Mutations in Extracellular Matrix Protein 1","volume":"16","author":"Chan","year":"2007","journal-title":"Exp. Dermatol."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"e13","DOI":"10.1002\/ski2.13","article-title":"Is Urinary Incontinence Associated with Lichen Sclerosus in Females? A Systematic Review and Meta-Analysis","volume":"1","author":"Kirby","year":"2021","journal-title":"Ski. Health Dis."},{"key":"ref_80","first-page":"571","article-title":"The Extracellular Matrix 1 Gene Is Essential for Early Mouse Development","volume":"272","author":"Sercu","year":"2005","journal-title":"FEBS J."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"6160","DOI":"10.4049\/jimmunol.0802664","article-title":"Tumor Regulatory T Cells Potently Abrogate Antitumor Immunity","volume":"182","author":"Liu","year":"2009","journal-title":"J. Immunol."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1868","DOI":"10.1053\/j.gastro.2005.03.043","article-title":"Peripheral and Intestinal Regulatory CD4+CD25high T Cells in Inflammatory Bowel Disease","volume":"128","author":"Maul","year":"2005","journal-title":"Gastroenterology"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"3639","DOI":"10.1210\/jc.2005-2337","article-title":"Regulatory T Cells in Human Autoimmune Thyroid Disease","volume":"91","author":"Marazuela","year":"2006","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"815","DOI":"10.1038\/sj.jid.5700174","article-title":"CD8+ T Cells Are Effector Cells of Contact Dermatitis to Common Skin Allergens in Mice","volume":"126","author":"Vocanson","year":"2006","journal-title":"J. Investig. Dermatol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.jaut.2010.03.004","article-title":"Amelioration of Experimental Autoimmune Encephalitis by Novel Peptides: Involvement of T Regulatory Cells","volume":"35","author":"Shapira","year":"2010","journal-title":"J. Autoimmun."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1097\/MED.0b013e328355a2d5","article-title":"Revisiting Regulatory T Cells in Type 1 Diabetes","volume":"19","author":"Zhang","year":"2012","journal-title":"Curr. Opin. Endocrinol. Diabetes. Obes."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1038\/ni904","article-title":"Foxp3 Programs the Development and Function of CD4+CD25+ Regulatory T Cells","volume":"4","author":"Fontenot","year":"2003","journal-title":"Nat. Immunol."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1111\/1751-2980.12296","article-title":"Genes Involved in the Regulation of Intestinal Permeability and Their Role in Ulcerative Colitis","volume":"16","author":"Prager","year":"2015","journal-title":"J. Dig. Dis."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"254","DOI":"10.5152\/tjg.2017.17043","article-title":"Extracellular Matrix Protein 1 Gene Rs3737240 Single Nucleotide Polymorphism Is Associated with Ulcerative Colitis in Turkish Patients","volume":"28","author":"Adali","year":"2017","journal-title":"Turk. J. Gastroenterol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"1054","DOI":"10.4049\/jimmunol.1502457","article-title":"Novel Function of Extracellular Matrix Protein 1 in Suppressing Th17 Cell Development in Experimental Autoimmune Encephalomyelitis","volume":"197","author":"Su","year":"2016","journal-title":"J. Immunol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"6055","DOI":"10.1038\/onc.2015.54","article-title":"ECM1 Regulates Tumor Metastasis and CSC-like Property through Stabilization of \u03b2-Catenin","volume":"34","author":"Lee","year":"2015","journal-title":"Oncogene"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/S0304-3835(03)00350-1","article-title":"Extracellular Matrix Protein 1 (ECM1) Is over-Expressed in Malignant Epithelial Tumors","volume":"200","author":"Wang","year":"2003","journal-title":"Cancer Lett."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/12\/12\/3070\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:35:18Z","timestamp":1760146518000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/12\/12\/3070"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,6]]},"references-count":92,"journal-issue":{"issue":"12","published-online":{"date-parts":[[2022,12]]}},"alternative-id":["diagnostics12123070"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics12123070","relation":{},"ISSN":["2075-4418"],"issn-type":[{"value":"2075-4418","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,12,6]]}}}