[Cutaneous T-cell lymphomas].

Abstract

Primitive cutaneous T-cell lymphomas are lymphomas clinically restricted to skin at diagnosis in opposite to skin localizations of systemic lymphomas. Cutaneous T-cell lymphomas are the most frequent. Beside mycosis fungoides and related forms, CD30+ lymphoproliferations (lymphomatoid papulosis, anaplastic large cell primitive cutaneous T-cell lymphomas) and erythrodermic T-cell lymphomas which constitute the main entities, other rare lymphomas are described: panniculitis like alpha/beta lymphoma, gamma/delta lymphoma, epidermotropic CD8+ aggressive lymphoma, small to medium T-cell lymphomas and acral CD8+ lymphoproliferations. Clinical skin examination and skin biopsy examination are crucial for diagnosis but recent advances in molecular genetics bring promising tools for diagnostic. Thanks to international cooperative groups, treatment of mycosis fungoides, CD30+ lymphoproliferations and erythrodermic lymphomas is now well established which is not the case for the other entities. Treatments may be classified in five categories: skin directed therapies, systemic non-chemotherapeutic treatments, immunomodulators, targeted immunotherapies and chemotherapies.