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The association of <jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content> with other systemic diseases, particularly neurocognitive diseases, provides a potential clue in the underlying pathogenesis of <jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content>. The role of <jats:italic>\n<jats:styled-content style=\"fixed-case\">HLA\u2010DQB1<\/jats:styled-content>*03:01<\/jats:italic> binding to the immunogenic portion of <jats:styled-content style=\"fixed-case\">BP180<\/jats:styled-content> provides a potential mechanism by which exposure to neuronal collagen <jats:styled-content style=\"fixed-case\">BP180<\/jats:styled-content> may lead to cutaneous disease. In our proposed multi\u2010hit hypothesis, patients with underlying neuronal disease are exposed to previously sequestered self\u2010antigen, most importantly <jats:styled-content style=\"fixed-case\">BP180<\/jats:styled-content>. Patients with the <jats:italic>\n<jats:styled-content style=\"fixed-case\">HLA\u2010DQB1<\/jats:styled-content>*03:01<\/jats:italic> allele show an increased T\u2010cell avidity to several epitopes of <jats:styled-content style=\"fixed-case\">BP180<\/jats:styled-content>, particularly the <jats:styled-content style=\"fixed-case\">BP180\u2010NC16a<\/jats:styled-content> domain. Thus, they have a genetic susceptibility to developing <jats:styled-content style=\"fixed-case\">BP<\/jats:styled-content> upon exposure to the target antigen. In a patient with dysregulation of Th1\/Th2 balance, anergy is lost and T\u2010cells are subsequently primed resulting in the development of functional autoimmunity against the <jats:styled-content style=\"fixed-case\">BP180\u2010NC16a<\/jats:styled-content> domain leading to clinically overt disease.<\/jats:p>","DOI":"10.1111\/tan.12960","type":"journal-article","created":{"date-parts":[[2017,1,19]],"date-time":"2017-01-19T14:13:51Z","timestamp":1484835231000},"page":"127-134","source":"Crossref","is-referenced-by-count":46,"title":["A multi\u2010hit hypothesis of bullous pemphigoid and associated neurological disease: Is <i><scp>HLA\u2010DQB1<\/scp>*03:01<\/i>, a potential link between immune privileged antigen exposure and epitope spreading?"],"prefix":"10.1111","volume":"89","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2906-2454","authenticated-orcid":false,"given":"K. 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