{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,11]],"date-time":"2026-03-11T23:35:20Z","timestamp":1773272120851,"version":"3.50.1"},"reference-count":12,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2015,2,26]],"date-time":"2015-02-26T00:00:00Z","timestamp":1424908800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2015,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Omenn syndrome is a combined immunodeficiency characterized by a generalized erythematous skin rash, enlarged lymph nodes, hepatosplenomegaly, severe susceptibility to infections, eosinophilia, and hyperimmunoglobulinemia E. A 3\u2010month\u2010old girl was admitted to our hospital with a history of recurrent sepsis. Physical examination revealed severe erythroderma, hepatosplenomegaly, lymphadenopathy, and failure to thrive. Laboratory findings revealed leukocytosis, lymphocytosis with high <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>3 T\u2010cells, a high <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4:<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>8 ratio, absence of <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>19 B\u2010cells, high eosinophil count, and low immunoglobulin levels. A heterozygote <jats:italic>RAG1<\/jats:italic> gene mutation was found. She had itchy, scaling, ichthyosiform erythroderma and protracted diarrhea. Cyclosporin treatment up to 10\u00a0mg\/kg effectively resolved erythroderma and lowered total eosinophil counts, and she gained weight during treatment. Since extensive erythroderma with generalized itching causes patient discomfort in Omenn syndrome, cyclosporin treatment can be considered while waiting for treatment with hematopoietic stem cell transplantation.<\/jats:p>","DOI":"10.1111\/pde.12522","type":"journal-article","created":{"date-parts":[[2015,2,26]],"date-time":"2015-02-26T07:00:10Z","timestamp":1424934010000},"source":"Crossref","is-referenced-by-count":6,"title":["Cyclosporin Treatment Improves Skin Findings in Omenn Syndrome"],"prefix":"10.1111","volume":"32","author":[{"given":"Sule","family":"Caglayan Sozmen","sequence":"first","affiliation":[{"name":"Division of Pediatric Allergy and Immunology Faculty of Medicine Dokuz Eylul University \u0130zmir Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sakine","family":"Isik","sequence":"additional","affiliation":[{"name":"Division of Pediatric Allergy and Immunology Faculty of Medicine Dokuz Eylul University \u0130zmir 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