{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T13:02:47Z","timestamp":1792501367605,"version":"4.5.0"},"reference-count":27,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2022,9,20]],"date-time":"2022-09-20T00:00:00Z","timestamp":1663632000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2023,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Paraneoplastic pemphigus (PNP) is a rare, often fatal, autoimmune blistering disease of the skin and mucous membranes. In children, PNP is frequently associated with Castleman disease (CD). This series describes five cases of PNP associated with CD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data were collected retrospectively from the medical records of patients with a diagnosis of PNP and CD from January 2013 to June 2022. Patients \u226422\u2009years old with clinical and immunopathologic evidence of PNP were included; CD was diagnosed histopathologically.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Two children, two adolescents, and one young adult (two males, three females) were included. The average age at disease presentation was 11.8\u2009years (range: 7\u201322\u2009years). Oral (<jats:italic>n<\/jats:italic>\u00a0=\u20095) and anogenital (<jats:italic>n<\/jats:italic>\u00a0=\u20093) mucositis were common. Four patients had \u201cunicentric\u201d CD (UCD); one patient had \u201cmulticentric\u201d CD (MCD). Castleman tumors were in the retroperitoneum (<jats:italic>n<\/jats:italic>\u00a0=\u20094) or axilla (<jats:italic>n<\/jats:italic>\u00a0=\u20091). One patient had myasthenia gravis without thymoma. Three patients had bronchiolitis obliterans (BO). Three patients had complete resection of their CD; two had partial resection. Three patients remain alive with a median follow\u2010up of 13\u2009months (range: 12\u2009months to 13\u2009years); two are clinically stable with resolution of mucocutaneous lesions; one has persistent BO requiring ongoing ventilatory support. Patients who remain alive had UCD with complete resection; all deceased patients had partial resection and BO.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Most patients had UCD, and the retroperitoneum was the most common location. Patients with MCD, incomplete resection, and BO died; patients with UCD and complete resection remain alive, even in the setting of BO. Consideration of PNP is critical when pediatric patients present with mucositis as PNP may be clinically indistinguishable from more common causes of mucositis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pde.15138","type":"journal-article","created":{"date-parts":[[2022,9,21]],"date-time":"2022-09-21T06:36:52Z","timestamp":1663742212000},"page":"90-95","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Paraneoplastic pemphigus associated with Castleman disease: A multicenter case series"],"prefix":"10.1111","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0382-4470","authenticated-orcid":false,"given":"Kelly K.","family":"Barry","sequence":"first","affiliation":[{"name":"Department of Dermatology Boston Children's Hospital, Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabella","family":"Plumptre","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of Massachusetts Chan Medical School  Worcester Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4299-400X","authenticated-orcid":false,"given":"Christopher G.","family":"Bazewicz","sequence":"additional","affiliation":[{"name":"Department of Dermatology Penn State Health Milton S. Hershey Medical Center  Hershey Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian P.","family":"Green","sequence":"additional","affiliation":[{"name":"Department of Dermatology Penn State Health Milton S. 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Dermatology University of Massachusetts Chan Medical School  Worcester Massachusetts USA"},{"name":"Department of Pediatrics University of Massachusetts Chan Medical School  Worcester Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marilyn G.","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Dermatology Boston Children's Hospital, Harvard Medical School  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,9,20]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/nejm199012203232503"},{"issue":"2","key":"e_1_2_9_3_1","first-page":"193","article-title":"Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus","volume":"137","author":"Nguyen 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