Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma (PCAECDTCL) is an extremely rare and aggressive subtype of cutaneous T-cell lymphoma. It is classified as a provisional entity among the primary cutaneous peripheral T-cell lymphomas. This condition is characterized by the proliferation of malignant CD8-positive cytotoxic T-cells that show a marked tendency to invade the epidermis (epidermotropism). The disease primarily affects the skin but can disseminate to other organs including the lungs, central nervous system, testes, and oral mucosa. Clinically, patients typically present with widespread, rapidly progressive skin lesions that may include localized or disseminated papules, nodules, plaques, and tumors. These lesions frequently undergo central necrosis and ulceration, which can be extensive and disfiguring. Hemorrhagic patches and superficial erosions resembling pagetoid reticulosis may also be observed. Unlike other forms of cutaneous T-cell lymphoma such as mycosis fungoides, this disease follows a highly aggressive clinical course with a poor prognosis. The median survival is generally reported to be less than three years. Treatment remains challenging and there is no established standard of care. Multiagent chemotherapy regimens similar to those used for aggressive non-Hodgkin lymphomas are commonly employed, though responses are often incomplete or short-lived. Allogeneic hematopoietic stem cell transplantation has been considered in selected cases. Radiation therapy may be used for localized disease or palliation. Given the rarity of this lymphoma, clinical trials and referral to specialized centers with expertise in cutaneous lymphomas are strongly recommended.

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