{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,8]],"date-time":"2026-02-08T17:00:48Z","timestamp":1770570048104,"version":"3.49.0"},"reference-count":18,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,4,10]],"date-time":"2025-04-10T00:00:00Z","timestamp":1744243200000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Experimental Dermatology"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Severe dermatitis, multiple allergies, and metabolic wasting syndrome (SAM) is a rare inherited disorder caused by biallelic loss\u2010of\u2010function mutations in the desmoglein\u20101 (<jats:italic>DSG1<\/jats:italic>) or desmoplakin (<jats:italic>DSP<\/jats:italic>) genes. Previous studies have demonstrated that acitretin and systemic biologics targeting IL\u201017, IL\u201012\/IL\u201023, and IL\u20104 are effective in treating SAM syndrome. We report the case of an 8\u2010year\u2010old girl diagnosed with SAM syndrome who suffered from recurrent rash episodes due to infections and achieved remission with a combination therapy of Spesolimab and acitretin. Comprehensive diagnostic workup, including serum inflammatory factor assays, flow cytometry, skin immunohistochemical staining, and skin RNA sequencing (RNA\u2010seq), revealed elevated IL\u201036G levels in SAM syndrome, which may be associated with the pathogenesis of generalised pustular psoriasis (GPP) through shared IL\u201036\u2010mediated mechanisms. This case highlights the therapeutic potential of targeting the IL\u201036 pathway in SAM syndrome and supports the use of skin RNA\u2010seq for personalised selection of anti\u2010inflammatory biologics in rare dermatological disorders. This report marks the first clinical application of Spesolimab in SAM syndrome, offering a novel therapeutic approach.<\/jats:p>","DOI":"10.1111\/exd.70097","type":"journal-article","created":{"date-parts":[[2025,4,10]],"date-time":"2025-04-10T09:44:39Z","timestamp":1744278279000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Clinical Responses and Transcriptomic Analysis of Spesolimab in a Girl With Severe Dermatitis, Multiple Allergies and Metabolic Wasting Syndrome"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-3733-1996","authenticated-orcid":false,"given":"Jinxiang","family":"Yang","sequence":"first","affiliation":[{"name":"Department of Dermatology Xinhua Hospital, Shanghai Jiaotong University School of Medicine  Shanghai China"},{"name":"Institute of Dermatology, Shanghai Jiaotong University School of Medicine  Shanghai 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