Abstract
Clinical practice guidelines for the management of cutaneous lymphomas have been prepared on behalf of the Japanese Dermatological Association and the Japanese Skin Cancer Society. Accurate diagnosis and staging are important, because treatment decisions depend primarily on the diagnosis and extent of the disease. Staging of the patients with mycosis fungoides (MF) and Sézary syndrome (SS) should be performed according to the revised TNMB classification system published in 2007. Treatment recommendations for each stage of MF/SS are summarized as a treatment algorithm. Because there have been few randomized controlled trials, most of the treatment recommendations are not based on high-level evidence. Local (skin-directed) therapies including topical corticosteroids, phototherapy, and radiation therapy are appropriate treatment options for early-stage MF. For refractory or more advanced cutaneous disease, systemic biological response modifiers such as oral retinoid and interferon may be combined with local therapies. Patients with advanced extracutaneous disease are treated with systemic chemotherapy, but long-term remissions are uncommon. [Skin Cancer (Japan) 2008; 23: 287-293]
