{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,2]],"date-time":"2025-08-02T00:11:32Z","timestamp":1754093492204,"version":"3.41.2"},"reference-count":15,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,6,27]],"date-time":"2025-06-27T00:00:00Z","timestamp":1750982400000},"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":["The Journal of Dermatology"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Angioedema is classified into mast cell\u2010mediated and bradykinin\u2010mediated types. Mast cell\u2010mediated angioedema (AE\u2010MC) frequently presents with wheals and may respond to antihistamines and\/or corticosteroids. In contrast, bradykinin\u2010mediated angioedema (AE\u2010BK) neither accompanies urticaria nor responds to these treatments, but may respond to medications targeting bradykinin or molecules involved in its production pathway. Hereditary angioedema (HAE), a subtype of AE\u2010BK, includes a small population of HAE that exhibit normal C1 inhibitor (C1\u2010INH) levels, classified as HAE with normal C1\u2010INH (HAE\u2010nC1INH). However, genetic abnormalities are not identified in most cases, complicating the differentiation from AE\u2010MC. We present a case of a 41\u2010year\u2010old woman with recurrent gastrointestinal edema with abdominal pain, and marked swelling of the abdomen and extremities without C1\u2010INH deficiency. Neither family history nor genetic mutations were identified; however, her symptoms, including the lack of urticaria, and resistance to antihistamines and corticosteroids were consistent with AE\u2010BK. Considering the possibility of AE\u2010BK including HAE\u2010nC1INH, she received intravenous plasma\u2010derived C1\u2010INH (pdC1\u2010INH) from Day 112 (with Day 0 as her first visit) when an attack occurred, resulting in a substantial reduction of symptom severity. From Day 266, she was treated with q4w 300\u2009mg omalizumab and her attack\u2010related symptoms largely reduced throughout the course without further administrations of pdC1\u2010INH. This case suggests a certain level of overlap between AE\u2010BK and AE\u2010MC, and the importance of trying anti\u2010immunoglobulin E medications for refractory angioedema.<\/jats:p>","DOI":"10.1111\/1346-8138.17838","type":"journal-article","created":{"date-parts":[[2025,6,27]],"date-time":"2025-06-27T08:14:24Z","timestamp":1751012064000},"page":"1330-1333","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["An Atypical Case of Angioedema With Normal <scp>C1<\/scp>\u2010Inhibitor Responding to Both <scp>C1<\/scp>\u2010Inhibitor Concentrate and Omalizumab"],"prefix":"10.1111","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3093-4694","authenticated-orcid":false,"given":"Koji","family":"Miyahara","sequence":"first","affiliation":[{"name":"Department of Internal Medicine Hiroshima City Hiroshima Citizens Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie","family":"Matsumoto","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Hiroshima City Hiroshima Citizens Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaki","family":"Kunihiro","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Hiroshima City Hiroshima Citizens Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoichiro","family":"Toi","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hiroshima City Hiroshima Citizens Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1569-6034","authenticated-orcid":false,"given":"Michihiro","family":"Hide","sequence":"additional","affiliation":[{"name":"Department of Dermatology Hiroshima City Hiroshima Citizens Hospital  Hiroshima Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,6,27]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.15214"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-021-08841-w"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/imr.12632"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.15090"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1808012"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/archinte.163.10.1229"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1572-0241.2006.00492.x"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-025-09027-4"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13601-015-0049-8"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjmed.2005.09.064"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1398-9995.2010.02496.x"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-016-0345-7"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/all.13083"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.3389\/fmed.2022.1048480"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2017.00846"}],"container-title":["The Journal of Dermatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/1346-8138.17838","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,1]],"date-time":"2025-08-01T12:57:21Z","timestamp":1754053041000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/1346-8138.17838"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,6,27]]},"references-count":15,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2025,8]]}},"alternative-id":["10.1111\/1346-8138.17838"],"URL":"https:\/\/doi.org\/10.1111\/1346-8138.17838","archive":["Portico"],"relation":{},"ISSN":["0385-2407","1346-8138"],"issn-type":[{"type":"print","value":"0385-2407"},{"type":"electronic","value":"1346-8138"}],"subject":[],"published":{"date-parts":[[2025,6,27]]},"assertion":[{"value":"2025-04-01","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-06-19","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-06-27","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}