{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T16:38:13Z","timestamp":1789749493633,"version":"4.0.1"},"reference-count":85,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,10,17]],"date-time":"2023-10-17T00:00:00Z","timestamp":1697500800000},"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":["Experimental Dermatology"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Pyoderma gangrenosum (PG) is a rare ulcerative neutrophilic dermatosis that is occasionally associated with primary immunodeficiency. Though contributions from dysregulation of the innate immune system, neutrophil dysfunction and genetic predisposition have been postulated, the precise pathogenesis of PG has not yet been elucidated. This article reviews reported cases of coexisting PG and primary immunodeficiency in order to gain insight into the complex pathophysiology of PG. Our findings suggest that variations in genes such as\n                    <jats:italic>RAG1<\/jats:italic>\n                    ,\n                    <jats:italic>ITGB2<\/jats:italic>\n                    ,\n                    <jats:italic>IRF2BP2<\/jats:italic>\n                    and\n                    <jats:italic>NF\u03baB1<\/jats:italic>\n                    might play a role in genetically predisposing patients to develop PG. These studies support the feasibility of the role of somatic gene variation in the pathogenesis of PG which warrants further exploration to guide targeted therapeutics.\n                  <\/jats:p>","DOI":"10.1111\/exd.14954","type":"journal-article","created":{"date-parts":[[2023,10,17]],"date-time":"2023-10-17T08:36:04Z","timestamp":1697531764000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["What can inherited immunodeficiencies reveal about pyoderma gangrenosum?"],"prefix":"10.1111","volume":"33","author":[{"given":"Yasmine","family":"Oprea","sequence":"first","affiliation":[{"name":"Albert Einstein College of Medicine  Bronx New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon","family":"Kody","sequence":"additional","affiliation":[{"name":"Department of Dermatology Oregon Health &amp; Science University  Portland Oregon USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hadir","family":"Shakshouk","sequence":"additional","affiliation":[{"name":"Department of Dermatology Oregon Health &amp; Science University  Portland Oregon USA"},{"name":"Department of Dermatology and Andrology, Alexandria Faculty of Medicine Alexandria University  Alexandria Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teri M.","family":"Greiling","sequence":"additional","affiliation":[{"name":"Department of Dermatology Oregon Health &amp; Science University  Portland Oregon USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen M.","family":"Anstey","sequence":"additional","affiliation":[{"name":"Department of Medicine, Section of Allergy and Clinical Immunology Oregon Health &amp; Science University  Portland Oregon USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex G.","family":"Ortega\u2010Loayza","sequence":"additional","affiliation":[{"name":"Department of Dermatology Oregon Health &amp; Science University  Portland Oregon USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,17]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.3389\/fgene.2021.673453"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01059"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-023-31914-z"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.2174\/138920210793175921"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-019-00737-x"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/ajd.13932"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ebiom.2023.104501"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/GOX.0000000000001886"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms21041402"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1990.tb06301.x"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2133.1998.02567.x"},{"issue":"2","key":"e_1_2_8_13_1","first-page":"119","article-title":"Pyoderma gangrenosum after bone marrow transplantation for leukocyte adhesion deficiency type 1","volume":"12","author":"Elenberg Y","year":"2010","journal-title":"Isr Med Assoc J"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2010.01260.x"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2010.01123.x"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.4103\/0019-5154.108076"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2013.12.887"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-015-0155-3"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.2340\/00015555-1929"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1612197"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/dth.12864"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13555-021-00507-x"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/pde.15292"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2020.04.019"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2022.02.008"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1038\/s12276-019-0227-1"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jid.2021.08.431"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1093\/ecco-jcc\/jjx158"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1111\/ddg.13173"},{"key":"e_1_2_8_30_1","unstructured":"SmithCIE BerglofA.X\u2010Linked Agammaglobulinemia. 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