{"title":"泛发性脓疱型银屑病患者伴有对静脉注射斯贝单抗无效的杂合子低表型 MPO 变异。","authors":"Youichi Ogawa, Eri Maejima, Takuya Takeichi, Takashi Okamoto, Hiroshi Mitsui, Shinji Shimada, Masashi Akiyama, Tatsuyoshi Kawamura","doi":"10.1111/1346-8138.17464","DOIUrl":null,"url":null,"abstract":"<p><p>Generalized pustular psoriasis (GPP) is a recurrent and sometimes life-threatening sterile pustular disease. Because interleukin (IL)-36 is the central cytokine in disease formation, spesolimab, which interferes with IL-36 receptor signaling, is highly effective. Here, we report a patient with GPP with a heterozygous hypomorphic MPO variant refractory to intravenous spesolimab. Although spesolimab showed excellent clinical efficacy in resolving pre-existing pustules and erythema, it did not suppress the emergence of new pustules and erythema, which did not decrease the peripheral blood neutrophil count, therefore bimekizumab, an anti-IL-17A/IL-17F antibody, was administered after the second spesolimab infusion, which resolved the pustules and erythema. We discuss the possible reasons for the resistance mechanism to spesolimab.</p>","PeriodicalId":94236,"journal":{"name":"The Journal of dermatology","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Generalized pustular psoriasis patient with a heterozygous hypomorphic MPO variant refractory to intravenous spesolimab.\",\"authors\":\"Youichi Ogawa, Eri Maejima, Takuya Takeichi, Takashi Okamoto, Hiroshi Mitsui, Shinji Shimada, Masashi Akiyama, Tatsuyoshi Kawamura\",\"doi\":\"10.1111/1346-8138.17464\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Generalized pustular psoriasis (GPP) is a recurrent and sometimes life-threatening sterile pustular disease. Because interleukin (IL)-36 is the central cytokine in disease formation, spesolimab, which interferes with IL-36 receptor signaling, is highly effective. Here, we report a patient with GPP with a heterozygous hypomorphic MPO variant refractory to intravenous spesolimab. Although spesolimab showed excellent clinical efficacy in resolving pre-existing pustules and erythema, it did not suppress the emergence of new pustules and erythema, which did not decrease the peripheral blood neutrophil count, therefore bimekizumab, an anti-IL-17A/IL-17F antibody, was administered after the second spesolimab infusion, which resolved the pustules and erythema. We discuss the possible reasons for the resistance mechanism to spesolimab.</p>\",\"PeriodicalId\":94236,\"journal\":{\"name\":\"The Journal of dermatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-09-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"The Journal of dermatology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1111/1346-8138.17464\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"The Journal of dermatology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1111/1346-8138.17464","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Generalized pustular psoriasis patient with a heterozygous hypomorphic MPO variant refractory to intravenous spesolimab.
Generalized pustular psoriasis (GPP) is a recurrent and sometimes life-threatening sterile pustular disease. Because interleukin (IL)-36 is the central cytokine in disease formation, spesolimab, which interferes with IL-36 receptor signaling, is highly effective. Here, we report a patient with GPP with a heterozygous hypomorphic MPO variant refractory to intravenous spesolimab. Although spesolimab showed excellent clinical efficacy in resolving pre-existing pustules and erythema, it did not suppress the emergence of new pustules and erythema, which did not decrease the peripheral blood neutrophil count, therefore bimekizumab, an anti-IL-17A/IL-17F antibody, was administered after the second spesolimab infusion, which resolved the pustules and erythema. We discuss the possible reasons for the resistance mechanism to spesolimab.