{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,27]],"date-time":"2026-06-27T02:41:28Z","timestamp":1782528088755,"version":"3.54.5"},"reference-count":8,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2015,3,17]],"date-time":"2015-03-17T00:00:00Z","timestamp":1426550400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2015,5]]},"abstract":"Abstract<\/jats:title>We report the case of a 2\u2010week\u2010old boy who presented with a vesiculopustular, bullous eruption in the setting of autoimmune enteropathy, hypothyroidism, membranous nephropathy, Coombs\u2010positive hemolytic anemia, and persistent eosinophilia. Immunologic testing revealed a deficiency of FOXP<\/jats:styled-content>3\u2010expressing regulatory T cells, and a diagnosis of immune dysregulation, polyendocrinopathy, enteropathy, X\u2010linked syndrome was made. Histologic analysis, immunofluorescence, and enzyme\u2010linked immunosorbent assay confirmed the bullous eruption as epidermolysis bullosa acquisita with associated collagen VII<\/jats:styled-content> autoantibody production. The skin lesions responded to systemic immunosuppressant therapy and have regressed after allogeneic bone marrow transplantation.<\/jats:p>","DOI":"10.1111\/pde.12550","type":"journal-article","created":{"date-parts":[[2015,3,17]],"date-time":"2015-03-17T08:24:40Z","timestamp":1426580680000},"source":"Crossref","is-referenced-by-count":10,"title":["Immune Dysregulation, Polyendocrinopathy, Enteropathy, X\u2010Linked Syndrome Associated with Neonatal Epidermolysis Bullosa Acquisita"],"prefix":"10.1111","volume":"32","author":[{"given":"Sabina","family":"Bis","sequence":"first","affiliation":[{"name":"Harvard Combined Dermatology Program Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheilagh M.","family":"Maguiness","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen E.","family":"Gellis","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynda C.","family":"Schneider","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pui Y.","family":"Lee","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luigi D.","family":"Notarangelo","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sevgi","family":"Keles","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Talal A.","family":"Chatila","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Birgitta A.","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Division of Immunology Department of Medicine Boston Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel D.","family":"Miller","sequence":"additional","affiliation":[{"name":"Department of Dermatology School of Medicine Boston University Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2015,3,17]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2008.08835.x"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000000029"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2012-100759"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.39.8.537"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.140.4.466"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2005.08.047"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(20000228)90:5<390::AID-AJMG9>3.0.CO;2-M"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2010.09.714"}],"container-title":["Pediatric Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpde.12550","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/pde.12550","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,1]],"date-time":"2023-10-01T13:04:56Z","timestamp":1696165496000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/pde.12550"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2015,3,17]]},"references-count":8,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2015,5]]}},"alternative-id":["10.1111\/pde.12550"],"URL":"https:\/\/doi.org\/10.1111\/pde.12550","archive":["Portico"],"relation":{},"ISSN":["0736-8046","1525-1470"],"issn-type":[{"value":"0736-8046","type":"print"},{"value":"1525-1470","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,3,17]]}}}