{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,15]],"date-time":"2026-09-15T13:56:06Z","timestamp":1789480566763,"version":"build-2803163510"},"reference-count":29,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2021,5,20]],"date-time":"2021-05-20T00:00:00Z","timestamp":1621468800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2021,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>PNP is a malignancy\u2010associated autoimmune mucocutaneous syndrome due to autoantibodies against plakins, desmogleins, and other components of the epidermis and basement membrane of epithelial tissues. PNP\u2010causing malignancies comprise mainly lymphoproliferative and hematologic neoplasms. PNP is extremely rare, especially in children.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Here, we present the first case of a child who developed PNP on a PTLD after small bowel transplantation because of a severe genetic protein\u2010losing enteropathy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The patient in this case report had a severe stomatitis, striate palmoplantar keratoderma, and lichenoid skin lesions. In addition, she had marked esophageal involvement. She had lung pathology due to recurrent pulmonary infections and ventilator injury. Although we found no evidence of BO, she died from severe pneumonia and respiratory failure at the age of 12\u00a0years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>It is exceptional that, despite effective treatment of the PTLD, the girl survived 5\u00a0years after her diagnosis of PNP. We hypothesize that the girl survived relatively long after the PNP diagnosis due to strong T\u2010cell suppressive treatments for her small bowel transplantation.<\/jats:p><\/jats:sec>","DOI":"10.1111\/petr.14023","type":"journal-article","created":{"date-parts":[[2021,5,20]],"date-time":"2021-05-20T10:14:43Z","timestamp":1621505683000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Paraneoplastic pemphigus associated with post\u2010transplant lymphoproliferative disorder after small bowel transplantation"],"prefix":"10.1111","volume":"25","author":[{"given":"Sander A. R.","family":"Fidder","sequence":"first","affiliation":[{"name":"Department of Pediatric Gastroenterology University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marieke C.","family":"Bolling","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gilles F. H.","family":"Diercks","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hendri H.","family":"Pas","sequence":"additional","affiliation":[{"name":"Department of Dermatology University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louise H. L.","family":"Hooimeijer","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura B.","family":"Bungener","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte W. 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