Cutaneous T cell lymphoma.

Cutaneous T cell lymphoma, previously known as mycosis fungoides, presents as either an aggressive disease (tumeur d'emblee type and the Sézary syndrome type) or a prolonged disease which evolves through patch, plaque, and tumor stages. Erythroderma is present either from the start or develops later in the disease process. Its incidence in the United States more than doubled from 1973 to 1984 (from 0.19 to 0.42 cases per 100,000 population). Staging classifications are being refined to reflect increasing knowledge of important prognostic indicators, e.g., absence or presence of lymph node involvement, pattern of lymph node involvement, and absence or presence of visceral disease. A number of newer treatment modalities is being investigated including the retinoids and ultraviolet A photopheresis.