{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T10:50:07Z","timestamp":1791370207934,"version":"4.3.1"},"reference-count":58,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T00:00:00Z","timestamp":1784592000000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000545","name":"British Association of Dermatologists","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000545","id-type":"DOI","asserted-by":"publisher"}]},{"name":"National Institute of Health Research Newcastle Biomedical Research Centre"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Ichthyoses (epidermal differentiation disorders) cause significant morbidity and mortality; however, national epidemiological data that link diagnoses of ichthyoses with systemic comorbidities are lacking.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To report epidemiological data on inherited ichthyoses in England, together with patient-level comorbidities and genetic testing status.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This national retrospective cohort study identified ichthyosis case records from healthcare databases in England, using International Classification of Diseases, 10th Revision (ICD-10) codes from 1998 to 2024. Cohort demographics, comorbidities, genetic testing data and mortality data were extracted from routinely collected National Health Service data.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We identified 4330 patients with ichthyosis, of whom 3758 were categorized as having a rare ichthyosis. The prevalence of rare ichthyoses was 51.6 per million (95% confidence interval 49.7\u201353.5). Compared with the reference population, the overall cohort was younger [median age 22 (interquartile range 11\u201349) vs. 41\u2005years], more likely to identify as Asian (17.1% vs. 9.6%; P &amp;lt; 0.001) and were more frequently in the most deprived quintiles (48.1% vs. 40.0%; P &amp;lt; 0.001). The rates of comorbidities, including asthma, inflammatory arthropathies and atrial fibrillation, were higher than in the reference population. Nineteen per cent of deaths occurred before the age of 25\u2005years compared with 1% of deaths at this age threshold in the reference population (P &amp;lt; 0.001). Genetic testing data revealed a low proportion of tested cases; pathogenic variants in genes known to cause ichthyosis were found in 56.3% of the tested cases (n = 90\/160).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>We report an increased range of comorbidities in patients living with rare ichthyoses, highlighting the systemic burden in patients categorized as having nonsyndromic ichthyosis. These data inform healthcare planning, research design and the redressing of inequities of care.<\/jats:p>\n                    <jats:p>An author video to accompany this article is available online.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/bjd\/ljag295","type":"journal-article","created":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T11:42:34Z","timestamp":1784374954000},"source":"Crossref","is-referenced-by-count":1,"title":["A national epidemiological study of inherited ichthyoses in England from 1998 to 2024"],"prefix":"10.1093","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4849-6618","authenticated-orcid":false,"given":"Mark D","family":"Eisner","sequence":"first","affiliation":[{"name":"Translational Clinical Research Institute, Newcastle University , Newcastle ,","place":["UK"]},{"name":"Department of Dermatology, Royal Victoria Infirmary , Newcastle ,","place":["UK"]},{"name":"National Disease Registration Service , Leeds 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,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeanette","family":"Aston","sequence":"additional","affiliation":[{"name":"National Disease Registration Service , Leeds ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-6290-715X","authenticated-orcid":false,"given":"Sally","family":"Vernon","sequence":"additional","affiliation":[{"name":"National Disease Registration Service , Leeds ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven","family":"Hardy","sequence":"additional","affiliation":[{"name":"National Disease Registration Service , Leeds ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0744-4985","authenticated-orcid":false,"given":"Shehnaz","family":"Ahmed","sequence":"additional","affiliation":[{"name":"British Association of Dermatologists , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-4911-7236","authenticated-orcid":false,"given":"Mandy","family":"Aldwin-Easton","sequence":"additional","affiliation":[{"name":"Ichthyosis Support Group , Wokingham ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3541-5858","authenticated-orcid":false,"given":"Shaimaa","family":"Lashin","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Nottingham University Hospitals NHS Foundation Trust , Nottingham ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Fordham","sequence":"additional","affiliation":[{"name":"Torbay and South Devon NHS Foundation Trust , Devon 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