Primary cutaneous T-cell lymphomas represent a wide variety of non-Hodgkin lymphomas that are characterized by a distinct clinical presentation. Advanced molecular and biological techniques have enhanced the recognition of cutaneous Tcell lymphomas. The most common subtypes of cutaneous T-cell lymphomas are the epidermotropic variants mycosis fungoides and Se´zary syndrome. At present, a stage-adjusted therapy is the best concept available, since early aggressive treatment options did not improve the prognosis of patients with cutaneous T-cell lymphomas. Accurate diagnostic and clinical assessment as well as identification of prognostic factors provides a helpful basis for treatment strategies. Current medical literature on diagnosis, prognosis, and treatment is reviewed with emphasis on new biologic response-modifying treatment options.

PRIMARY CUTANEOUS T-cell lymphomas (CTCL) represent a heterogeneous group of lymphomas with a distinct clinical presentation, behaviour, and prognosis.