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This study describes the clinical and biological characteristics of UCD\u2010PNP patients in a large Western cohort. A total of 148 patients diagnosed with UCD were identified, including 14 patients with a defined PNP. PNP was significantly associated with myasthenia gravis (MG) and FDC sarcoma during follow\u2010up (FDCS). PNP was also significantly associated with reduced survival. These data, together with a multivariate analysis by principal components, led to the identification of UCD\u2010PNP as a group at risk of MG, FDCS and death. <jats:italic>PDGFRB<\/jats:italic> sequencing performed on UCD lesions from six patients found the gain\u2010of\u2010function p.N666S variant in two. Interestingly, both patients had hyaline\u2010vascular UCD subtype, were in the UCD\u2010PNP subgroup and had FDCS. Sera from 25 UCD\u2010PNP patients and 6 PNP patients without UCD were tested for PNP\u2010associated autoantibodies. Sera from UCD\u2010PNP patients had a strong reactivity against the N\u2010terminal domain of recombinant periplakin (rPPL, 82%) and showed reactivity against at least two domains of rPPL. These features were not found in patients with UCD alone or in the PNP group without UCD. These data indicate that UCD\u2010PNP patients belong to a subgroup sharing strong clinical and biological identity that might help to decipher the different dynamics of UCD natural history.<\/jats:p>","DOI":"10.1111\/bjh.18847","type":"journal-article","created":{"date-parts":[[2023,5,24]],"date-time":"2023-05-24T02:40:25Z","timestamp":1684896025000},"page":"267-278","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Paraneoplastic pemphigus uncovers distinct clinical and biological phenotypes of western unicentric Castleman disease"],"prefix":"10.1111","volume":"202","author":[{"given":"Yannick","family":"Dieudonn\u00e9","sequence":"first","affiliation":[{"name":"Department of Clinical Immunology and Internal Medicine, National Reference Centre for Systemic Autoimmune Diseases (CNR RESO) Strasbourg University Hospital  Strasbourg France"},{"name":"INSERM UMR\u2010S1109 Universit\u00e9 de Strasbourg  Strasbourg 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