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.
Table Graphic Jump Location Table 112-1Mycosis Fungoides Treatment Table ||Download (.pdf) Table 112-1 Mycosis Fungoides Treatment Table
|MEDICATION NAME ||INDICATIONb ||MECHANISM OF ACTION ||DOSING ||ADVERSE EFFECTS ||SUGGESTED MONITORING ||LEVEL OF EVIDENCE (REFERENCE) |
|Skin-Directed Tx |
|Topical carmustine ||LSD, GSD ||alkylating drug, carbamylation of amino acids in protein ||Solution (10 mg/60 mL water), ointment (10 mg/100 g petrolatum) ||Alopecia, burning sensation of skin, hyperpigmentation ||Clinical response ||III1 |
|Topical corticosteroids ||LSD ||Anti-inflammatory, antimitotic, immunosuppressive effects ||Class I-IV cream/ointment/lotion; apply QD ||Skin atrophy, striae, rosacea, perioral dermatitis, acne, and purpura ||None ||III2-4 |
|Topical imiquimod ||LSD ||Toll-like receptor 7 activator ||Different concentrations, different regimens ||Skin irritation (>10%), flu-like symptoms ||Clinical response ||III5-7 |
|Topical mechlorethamine (nitrogen mustard) ||LSD, GSD (2nd IA, IBa) ||Alkylating drug ||0.016% gel; apply QD ||Skin irritation (>10%), allergic contact dermatitis ||Clinical response ||IB8,9 |
|Topical retinoids (bexarotene) ||LSD (2nd: IA, IBa) ||Targets retinoid X receptor (anti-inflammatory, antiproliferative) ||1% gel; apply to lesions every other day for 1 wk initially, then increase qwk up to q6h ||Skin irritation ||Clinical response ||IIB10,11 |
|Phototherapy (UVB, NB-UVB, PUVA, UVA1) ||LSD, GSD ||Inducing apoptosis, cytotoxic effects, influences dermal Langerhans cell function ||Different protocols for initiation, consolidation, and maintenance phases12,13 ||Erythema, pruritus, sunburn, secondary skin cancer ||Clinical response ||IB14,15 |
|Local radiation ||LSD ||DNA damage ||8-12 Gy; 24-30 Gy for unilesional presentation ||Dermatitis ||None ||IV |
|TSEBT ||GSD ||DNA damage ||12 Gy ||Skin irritation, erythema, dermatits, hair loss, nail loss, fatigue ||None ||IIA16,17 |
|Systemic Therapy |
|Brentuximab vedotin ||2nd: CD30+ MFa ||ADC-CD30 complex || |
1.8 mg/kg up to a max. of 180 mg every 3 wk ...