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J Investig Med High Impact Case Rep ; 12: 23247096241253337, 2024.
Article de Anglais | MEDLINE | ID: mdl-38742532

RÉSUMÉ

Subcutaneous panniculitis-like T-cell lymphoma (SPTLP), a unique variant of primary cutaneous T-cell lymphomas, clinically mimics subcutaneous panniculitis. It is typified by the development of multiple plaques or subcutaneous erythematous nodules, predominantly on the extremities and trunk. Epidemiological findings reveal a greater incidence in females than males, affecting a wide demographic, including pediatric and adult cohorts, with a median onset age of around 30 years. Diagnosis of SPTLP is complex, hinging on skin biopsy analyses and the identification of T-cell lineage-specific immunohistochemical markers. Treatment modalities for SPTLP are varied; while corticosteroids may be beneficial initially for many patients, a substantial number require chemotherapy, especially in cases of poor response or relapse. Generally, SPTLP progresses slowly, yet approximately 20% of cases advance to hemophagocytic lymphohistiocytosis (HLH), often correlating with a negative prognosis. We report a case of a young male patient presenting with prolonged fever, multiple skin lesions accompanied by HLH, a poor clinical course, and eventual death, diagnosed postmortem with SPTLP. In addition, we also present a literature review of the current evidence of some updates related to SPTLP.


Sujet(s)
Lymphohistiocytose hémophagocytaire , Lymphome T , Panniculite , Humains , Mâle , Biopsie , Diagnostic différentiel , Issue fatale , Lymphohistiocytose hémophagocytaire/anatomopathologie , Lymphohistiocytose hémophagocytaire/diagnostic , Lymphohistiocytose hémophagocytaire/complications , Lymphome T/anatomopathologie , Lymphome T/complications , Lymphome T/diagnostic , Lymphome T cutané/anatomopathologie , Lymphome T cutané/complications , Lymphome T cutané/diagnostic , Panniculite/anatomopathologie , Panniculite/diagnostic , Peau/anatomopathologie , Tumeurs cutanées/anatomopathologie , Tumeurs cutanées/complications , Jeune adulte
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