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Mycosis fungoides is a rare subtype of cutaneous T-cell lymphoma in which clones of a malignant transformed T lymphocyte accumulate primarily in the skin. The degree of infiltration and inflammation leads to the typical clinical presentation with patches, plaques, and/or tumors. An erythrodermic presentation or involvement of lymph nodes and visceral organs is also possible in further stages. The course of the disease is often indolent, and progression is mostly slowly. Previous studies have already shown that less aggressive therapies can lead to more sustainable responses. Therefore, therapeutic approaches should be stage dependent. In early stage-disease and limited skin involvement skin-directed therapies (SDT) are recommended. In early-stage disease with higher skin disease burden the addition of systemic therapeutics can be considered. For advanced-stage disease (≥IIB) systemic therapies are recommended. SDT can be added at any stage. Single lesions can be treated with local radiation therapy. For example, for stage 1A/B disease, if the patient is recalcitrant to topical steroids and phototherapy, the authors typically prescribe topical mechlorethamine for limited skin disease and Bexarotene or low-dose methotrexate for generalized skin disease, as systemic therapies with mogamulizumab or brentuximab vedotin are usually reserved for more recalcitrant cases. See Table 112-1.
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