Primary cutaneous B‑cell lymphomas are rarely encountered and represent 25% of all cutaneous lymphomas. Follicular B‑cell lymphoma and marginal zone lymphoma belong to indolent subtypes which as a rule have no systemic dissemination and, thus, a mostly unchanged life expectancy. Therefore, skin-directed treatment options such as excision or radiotherapy are usually sufficient to control the disease. In contrast, cutaneous diffuse large B‑cell lymphoma and EBV-associated B‑cell lymphomas of the skin belong to more aggressive entities which demand a systemic first-line upfront therapy with R‑CHOP. Nevertheless, mortality is still high and comparable to that of systemic/nodal large B‑cell lymphomas so that the identification of pathogenetic driver mutations or novel therapeutic targets may pave the way to better target-oriented therapies.
Keywords: Follicle center cell lymphoma; Follicular B‑cell lymphoma; Histology; Immunochemotherapy; Marginal zone lymphoma.