Multiple cutaneous ulcers revealing a primary cutaneous Epstein‐Barr virus‐positive diffuse large B‐cell lymphoma

Abstract

Primary cutaneous Epstein-Barr virus-positive diffuse large B-cell lymphoma (PC-EBV-DLBCL) is an exceptional and aggressive neoplasia with a poorer prognosis than other cutaneous lymphoma. This case highlights this immunosenescence-associated lymphoid malignancy as an edifying cause of multiple skin ulcerations, distinct from other leg ulcers unrelated to arterial and venous disease. Approximately, 20% of cutaneous lymphomas are B-lymphocyte-derived malignancies exclusively involving cutaneous site at the time of diagnosis. Cutaneous B-cell lymphomas (CBCL) are classified into five distinct subgroups: primary cutaneous follicle center lymphoma (PCFCL); primary cutaneous marginal zone lymphoma (PCMZL); primary cutaneous diffuse large B-cell lymphoma (PC-DLBCL), leg type; intravascular diffuse large B-cell lymphoma (IV-DLBCL); and DLBCL, not otherwise specified (NOS). The prognosis of CBCL may vary depending on the subtype of CBCL. PCMZL and PCFCL are indolent forms, whereas PCLBCL, leg type, and IV-DLBCL have an intermediate to aggressive clinical course. The 2016 revision of the World Health Organization (WHO) classification of lymphoid malignancies recognized primary cutaneous Epstein-Barr virus (EBV)-positive DLBCL as a distinct entity within the group of "DLBCL NOS". 1 This is a rare but aggressive type of cutaneous lymphoma, which predominantly affects elderly and/or immunodeficient patients. 2 However, this entity must be distinguished from EBV-positive mucocutaneous ulcers (MCU)