Linfoma primario cutáneo agresivo epidermotrópico de células T citotóxicas CD8 positivo: Caso clínico

Abstract

Primary cutaneous aggressive epidermotropic cytotoxic CD8 positive T cell lymphoma, is an uncommon disease, with an aggressive clinical behavior. Differentiation with other types of cutaneous T-cell lymphoma (CTCL) that express a CD8+ cells, is based only on clinical grounds and in certain morphological characteristics, such as a marked epidermotropism with squamous cell necrosis. We report a 50-year-old male presenting with painless cutaneous lesions appearing in trunk, limbs, scalp and face, suggestive of cutaneous lymphoma. He was admitted to the hospital in bad conditions, with confluent papules and tumors, some of them ulcerated and with foul smelling honey-colored crusts, involving the complete body surface. Cutaneous biopsy demonstrated a CD8 positive epidermotropic cytotoxic T cell lymphoma. He was treated with chemotherapy with an excellent initial response, but cutaneous lesions reappeared after four cycles. He did not respond to rescue chemotherapy and died seven months after diagnosis.

Probable Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-cell Lymphoma: A Case Report of a Diagnostic Challenge

“…Presentation and clinical behavior distinguish this cutaneous lymphoma from other cutaneous lymphomas. CD8+ mycosis fungoides [ 8 , 9 ], gamma-delta mycosis fungoides, lymphomatoid papulosis subtype D, and primary cutaneous T-cell lymphoma, not otherwise specified (NOS) should all be considered [ 5 ].…”

Nasolabial Ulcerated Placard Revealing a Primary Cutaneous Aggressive Epidermotropic CD8+ T-cell Lymphoma

“…Treatment is not well codified and is based on multidrug therapy with different protocols: CHOP (Endoxan, doxorubicin, vincristine, onset, and Isone), CHOEP (Endoxan, doxorubicin, vincristine, onset, etoposide, and Isone), hyper CVAD (Endoxan, vincristine, Adriamycin, and dexamethasone). The response is unsatisfactory and usually partial [ 1 , 9 ]. Partial or complete responses have been reported in the literature with electron therapy [ 9 , 10 ].…”

“…The response is unsatisfactory and usually partial [ 1 , 9 ]. Partial or complete responses have been reported in the literature with electron therapy [ 9 , 10 ]. Stem cell transplantation with or without chemotherapy or anti-CD 52 monoclonal antibody has been reported in the literature with partial efficacy [ 1 , 3 , 10 ].…”