{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,14]],"date-time":"2026-02-14T01:15:17Z","timestamp":1771031717626,"version":"3.50.1"},"reference-count":19,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T00:00:00Z","timestamp":1765324800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T00:00:00Z","timestamp":1765324800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Cutan Pathol"],"published-print":{"date-parts":[[2026,3]]},"abstract":"ABSTRACT<\/jats:title>\n \n Primary cutaneous aggressive epidermotropic CD8\n +<\/jats:sup>\n cytotoxic T\u2010cell lymphoma (PCAECTCL) is a rare, rapidly progressive cutaneous T\u2010cell lymphoma that poses significant diagnostic challenges, particularly in mucocutaneous variants. We report a case in a 49\u2010year\u2010old man who presented with upper lip swelling initially misdiagnosed as angioedema and cheilitis granulomatosa. A lip biopsy revealed atypical CD4\/CD8 double\u2010negative T cells with marked epidermotropism, initially interpreted as pagetoid mycosis fungoides. Subsequent immunophenotyping demonstrated a cytotoxic profile (CD3\n +<\/jats:sup>\n , CD2\n +<\/jats:sup>\n , CD7\n +<\/jats:sup>\n , CD5\n \u2212<\/jats:sup>\n , CD4\n \u2212<\/jats:sup>\n , CD8\n +<\/jats:sup>\n , CD20\n +<\/jats:sup>\n , perforin\n +<\/jats:sup>\n , granzyme B\n +<\/jats:sup>\n , TCRBF1\n +<\/jats:sup>\n , CD56\n \u2212<\/jats:sup>\n , and TCR\u03b4\n \u2212<\/jats:sup>\n ). Imaging later identified tongue involvement with a similar phenotype, though CD8 expression was weak and CD20 expression was absent. The disease progressed to lymph nodes, oropharynx, and stomach, with an eventual phenotypic switch to CD8\n +<\/jats:sup>\n . Molecular testing confirmed similar clonal T\u2010cell rearrangements across all sites. Sequencing identified JAK3 and TP53 mutations, while chromosomal microarray revealed recurrent losses (1p, 2q, 3p, 4p, 11q, 13q, and 17q) and gains (7q, 17p), without JAK2 fusion. Despite aggressive therapy, the patient died 21\u2009months after diagnosis. This case delineates the importance of early biopsy, integration of molecular studies, and the urgent need for therapies to improve outcomes in this aggressive lymphoma.\n <\/jats:p>","DOI":"10.1111\/cup.70035","type":"journal-article","created":{"date-parts":[[2025,12,10]],"date-time":"2025-12-10T23:43:26Z","timestamp":1765410206000},"page":"263-269","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["An Unusual Case of Primary Mucocutaneous Cytotoxic T\u2010Cell Lymphoma With Epidermotropism and Phenotype Switch: Diagnostic and Molecular Insights"],"prefix":"10.1111","volume":"53","author":[{"given":"Amrit P.","family":"Singh","sequence":"first","affiliation":[{"name":"Department of Pathology University of Virginia Health Charlottesville Virginia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7716-6680","authenticated-orcid":false,"given":"Ifeyinwa E.","family":"Obiorah","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Virginia Health Charlottesville Virginia USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,12,10]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)65144-9"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2018-11-881268"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.12371"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2016.240"},{"issue":"3","key":"e_1_2_10_6_1","first-page":"229","article-title":"Primary Bone Marrow CD8 Cytotoxic T\u2010Cell Lymphoma Coexpressed CD20: A Case Report and Literatures Review","volume":"34","author":"Jin X.","year":"2013","journal-title":"Zhonghua Xue Ye Xue Za Zhi = Zhonghua Xueyexue 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