Primary cutaneous B-cell lymphomas are a heterogeneous group of B-cell lymphomas that present in the skin without evidence of extracutaneous disease at the time of diagnosis. In recent classifications three main types of cutaneous B-cell lymphomas are recognised: primary cutaneous marginal zone lymphoma, primary cutaneous follicle center lymphoma and primary cutaneous large B-cell lymphoma, leg type. In this review, the main characteristics and practice guidelines for the management and treatment of these three types of cutaneous B-cell lymphomas are presented. Other types of B-cell lymphomas that can present with skin-limited disease, such as intravascular large B-cell lymphoma, B-lymphoblastic lymphoma and immunodeficiency-associated B-cell proliferations will shortly be discussed.

(BELG J HEMATOL 2017;8(6):213–21)