CD8+ aggressive cutaneous epidermotropic T lymphoma (LCAE) is a rare and poorly characterized variant, still considered to be a temporary entity according to the WHO (World Health Organization) classification of 2017. About twenty cases have been reported in the literature (2, 3.4).
Clinically, it presents as ulcerated, necrotic, sometimes hemorrhagic plaques, nodules or tumors, which develop rapidly and aggressively, giving rise to extra-cutaneous damage. Our observation illustrates a clinical presentation also associating a quasi-erythroderma, and a palmoplantar keratoderma (4.9).
Histologically, the infiltrate is composed of lymphocytes of varying sizes. Epidermotropism is constant. Skin immunostaining shows tumor CD8+T lymphocytes expressing cytotoxic proteins, which probably explains the local and general aggressiveness of the disease, the angiodestructive nature of the infiltrate and the necrotic lesions (2, 3, 9).
The differential diagnosis arises with other indolent cutaneous T lymphomas, mainly pagetoid reticulosis and CD8+ mycosis fungoides (8).
There is no standardized treatment to date. The first-line treatment generally involves the CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) or EPOCH (Etoposide, Prednisone, Oncovin, Cyclophosphamide hydrochloride dexorubicin) protocols, but generally results in treatment failure. Recent studies suggest switching to second-line therapies such as gemcitabine, pralatrexate, brentuximab, as well as allogeneic stem cell transplantation, which may have allowed complete remission in a few rare cases (5, 6, 7). Recurrence is the rule with a median survival not exceeding 34 months (4, 8, 9)