{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T19:22:59Z","timestamp":1792005779484,"version":"4.4.0"},"reference-count":45,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,2,25]],"date-time":"2025-02-25T00:00:00Z","timestamp":1740441600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2025,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background\/Objectives<\/jats:title>\n                    <jats:p>Ichthyosis prematurity syndrome (IPS) is a rare autosomal recessive congenital disorder characterized\u00a0by premature birth, neonatal respiratory distress, eosinophilia, and a thick, clay\u2010like vernix at birth. This review aims to summarize the available reported cases of IPS, including genetic etiology, clinical features, management, and prognosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A search was conducted using MEDLINE, Embase, Scopus, Web of Science, and CINAHL, from inception to September 3, 2024 using the search terms \u201cIPS\u201d or \u201cichthyosis congenita type IV\u201d. This systematic review was registered on the PROSPERO database (CRD42024533988). Any publication reporting a case of IPS was suitable for inclusion. Quality appraisal was performed according to CARE guidelines. Descriptive statistical analysis was performed using R v4.3.1 (R Foundation, Vienna, Austria).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We identified 26 studies reporting 59 cases of IPS (PROSPERO database: CRD42024533988). While IPS was previously thought to predominantly affect Norwegian and Swedish patients, we identified cases from a broad range of ethnicities. The median duration of the cutaneous scale was 10\u2009weeks, though patients usually have persisting atopy. Long\u2010term prognosis is generally good, although six (10.2%) fulminant cases were identified with an estimated 6\u2010month survival rate of 87.6% (95% confidence interval: 78.8\u201397.4). Mortality in these cases was attributed to respiratory and\/or cardiac sequelae or sepsis. Limitations include heterogeneity in the reporting of cases and low quality of data in several aspects of data collection.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The cutaneous manifestations of IPS resolve over a period of weeks, and patients generally have a favorable long\u2010term prognosis. However, rare cases of death and other complications have been reported.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pde.15909","type":"journal-article","created":{"date-parts":[[2025,2,26]],"date-time":"2025-02-26T00:48:11Z","timestamp":1740530891000},"page":"747-753","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Ichthyosis Prematurity Syndrome: A Systematic Review of the Literature"],"prefix":"10.1111","volume":"42","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-3201-9573","authenticated-orcid":false,"given":"Grace X.","family":"Li","sequence":"first","affiliation":[{"name":"Faculty of Medicine and Health University of New South Wales  Kensington Australia"},{"name":"Department of Dermatology Liverpool Hospital  Liverpool Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn","family":"Chen","sequence":"additional","affiliation":[{"name":"School of Medicine Western Sydney University  Campbelltown Australia"},{"name":"Ingham Institute for Applied Medical Research  Sydney Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deshan F.","family":"Sebaratnam","sequence":"additional","affiliation":[{"name":"Faculty of Medicine and Health University of New South Wales  Kensington Australia"},{"name":"Department of Dermatology Liverpool Hospital  Liverpool Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James P.","family":"Pham","sequence":"additional","affiliation":[{"name":"Faculty of Medicine and Health University of New South Wales  Kensington Australia"},{"name":"Department of Dermatology Liverpool Hospital  Liverpool Australia"},{"name":"Ingham Institute for Applied Medical Research  Sydney Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,2,25]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2008.06.014"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/pde.12320"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2009.06.021"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jdermsci.2012.11.593"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M112.416990"},{"key":"e_1_2_9_7_1","article-title":"Ichthyosis Prematurity Syndrome With Separation of Fetal Membranes and Neonatal Asphyxia","volume":"2012","author":"Dereksson K.","year":"2012","journal-title":"British Medical Journal Case Reports"},{"issue":"9","key":"e_1_2_9_8_1","first-page":"1055","article-title":"Ichthyosis Prematurity Syndrome: From Fetus to Adulthood","volume":"152","author":"Lwin S. 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