Abstract
The management of patients with primary cutaneous lymphomas requires first of all a diagnostic work-up in order to differentiate between patients with nodal non-Hodgkin lymphomas with cutaneous manifestations and those with primary cutaneous lymphomas. In the latter an exact diagnosis of the type of cutaneous lymphoma has to be determined due to varying prognoses between the types of primary cutaneous lymphomas. In general, cutaneous lymphoma patients in early stages of disease can be treated successfully with skin-directed therapies. More advanced stages and some aggressive types like primary cutaneous diffuse large B-cell lymphoma, leg type or the leukemic variant of cutaneous T-cell lymphoma, the Sézary syndrome, require systemic treatment. The current recommendations regarding systemic therapy of patients with primary cutaneous lymphomas are reviewed.
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