Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare entity representing less than 1% of cutaneous lymphomas. It has an aggressive clinical manifestation with a poor prognosis. It is characterized by cytotoxic and epidermotropic CD8+ proliferation. It also expresses the TIA-1 marker. We report a new case for its display and aggressive character, diagnostic difficulty, and good therapeutic response to chemotherapy. This is a 62-year-old female patient admitted to the hospital for a nasolabial ulcerated placard evolving for two years. Clinical examination revealed submandibular lymph nodes. The specimen analysis associated with anatomoclinical manifestation was concluded for a primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. Tumor extent assessment did not show any secondary localization. The blood tests and serology were unremarkable. The patient had benefited from a CHOEP-type multidrug therapy protocol with complete healing of the lesion after three courses of chemotherapy.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9381253 | PMC |
| http://dx.doi.org/10.1155/2022/5672783 | DOI Listing |
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Article Synopsis
- Primitive cutaneous T-cell lymphomas are skin-specific lymphomas, distinct from systemic lymphomas affecting the skin.
- The most common types include mycosis fungoides, CD30+ lymphoproliferations, and erythrodermic T-cell lymphomas, with several rarer forms also identified.
- Diagnosis relies on clinical skin exams and biopsies, enhanced by molecular genetics, while treatments are categorized into five main types, including skin therapies and immunotherapies.
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