The diagnosis of primary cutaneous B-cell lymphoma (CBCL) requires that the search for a more widespread lymphoma has been negative. The clinical presentation, outlook, and treatment options of the common types of CBCLs, with emphasis on differences or similarities to their nodal counterparts, are discussed. Treatment may range from observation to topical therapies to systemic therapies, depending on the histology, degree and area of skin involvement, patient performance, and comorbidities. Rare lymphomas, such as intravascular large B-cell lymphoma and Epstein-Barr virus-positive cutaneous lymphoproliferations that are associated with immunodeficiency, are also briefly described.
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