{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T02:23:18Z","timestamp":1785291798276,"version":"3.55.0"},"reference-count":30,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2021,6,5]],"date-time":"2021-06-05T00:00:00Z","timestamp":1622851200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Most cases of Stevens\u2013Johnson syndrome and toxic epidermal necrolysis are drug\u2010induced. A small subset of cases remain with unknown aetiology (idiopathic epidermal necrolysis [IEN]).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>We sought to better describe adult IEN and understand the aetiology.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective study was conducted in 4 centres of the French national reference centre for epidermal necrolysis. Clinical data were collected for the 19 adults hospitalized for IEN between January 2015 and December 2019. Wide toxicology analysis of blood samples was performed. Histology of IEN cases was compared with blinding to skin biopsies of drug\u2010induced EN (DIEN, \u2018controls\u2019). Available baseline skin biopsies were analysed by shotgun metagenomics and transcriptomics and compared to controls.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      IEN cases represented 15.6% of all EN cases in these centres. The median age of patients was 38 (range 16\u201351) years; 68.4% were women. Overall, 63.2% (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a012) of cases required intensive care unit admission and 15.8% (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a03) died at the acute phase. Histology showed the same patterns of early\u2010 to late\u2010stage EN with no difference between DIEN and IEN cases. One toxicology analysis showed unexpected traces of carbamazepine; results for other cases were negative. Metagenomics analysis revealed no unexpected pathological microorganism. Transcriptomic analysis highlighted a different pro\u2010apoptotic pathway in IEN compared to DIEN, with an overexpression of apoptosis effectors TWEAK\/TRAIL.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>IEN affects young people and is a severe form of EN. A large toxicologic investigation is warranted. Different pathways seem involved in IEN and DIEN, leading to the same apoptotic effect, but the primary trigger remains unknown.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/jdv.17274","type":"journal-article","created":{"date-parts":[[2021,4,8]],"date-time":"2021-04-08T21:25:39Z","timestamp":1617917139000},"page":"1569-1576","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Towards a better understanding of adult idiopathic epidermal necrolysis: a retrospective study of 19 cases"],"prefix":"10.1111","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9077-1228","authenticated-orcid":false,"given":"P.","family":"Monnet","sequence":"first","affiliation":[{"name":"Dermatology Department AP\u2010HP Henri Mondor Hospital Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Microbiology Department AP\u2010HP Henri Mondor Hospital Cr\u00e9teil France"},{"name":"INSERM U955 Universit\u00e9 Paris Est Cr\u00e9teil Val de Marne UPEC Cr\u00e9teil France"},{"name":"Reference Center for Toxic Bullous Diseases and Severe Drug Reactions TOXIBUL Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O.","family":"Gaudin","sequence":"additional","affiliation":[{"name":"Dermatology Department AP\u2010HP Henri Mondor Hospital Cr\u00e9teil France"},{"name":"Reference Center for Toxic Bullous Diseases and Severe Drug Reactions TOXIBUL Cr\u00e9teil France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Cirotteau","sequence":"additional","affiliation":[{"name":"Dermatology Department Saint Andr\u00e9 Hospital Bordeaux France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.","family":"Papouin","sequence":"additional","affiliation":[{"name":"Pathology Department AP\u2010HP Henri Mondor Hospital Cr\u00e9teil 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