Outline of the Treatment of Mycosis Fungoides

Abstract

The early identification, diagnosis and management of Mycosis fungoides is imperative in a dermatology out patient setting. This activity reviews the etiolopathogenesis, epidemiology, clinical and histological features, diagnosis and treatment of Mycosis fungoides. For patients with early-stage mycosis fungoides (IIA or below), the various treatment options are topical corticosteroids, topical nitrogen mustards (mechlorethamine, HN2), topical bexarotene, imiquimod, psoralen-ultraviolet A (PUVA) therapy or ultraviolet B (UVB) therapy.  Local radiation therapy may be used for localized lesions (such as in pagetoid reticulosis). Systemic therapies, such as retinoids or interferons, histone deacetylase (HDAC) inhibitors, or low-dose methotrexate are used if skin-directed therapies fail, if skin symptoms are extensive/severe, or if patients have a worse prognostic profile such as folliculotropic mycosis fungoides, large cell transformation, or early blood involvement. Advanced stage (IIB to IV) mycosis fungoides is a heterogeneous group that encompasses those patients that present with extracutaneous disease or advanced skin lesions (e.g., tumors). It is often a chronic or persistent disease with a relapsing course. The main goals of therapy are long-term disease control, prompt symptom relief, and management of life-threatening (aggressive) disease. For patients with generalized tumors, total skin electron beam therapy (TSEBT) or systemic therapies are acceptable treatment options. TSEBT can be followed by other skin-directed therapies or systemic therapies to prolong response duration. For more extensive disease, combinations of skin-directed therapies and systemic therapies are used. The various systemic options available include methotrexate, bexarotene, targeted immunotherapy (such as alemtuzumab), polychemotherapy.