RESUMO
Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, characterized by skin-homing CD4+ T cells derivation, indolent course, and low-grade of malignancy. Mycosis fungoides's classic type typically onsets with cutaneous erythematous patches, plaque, and tumor. In WHO-EORTC classification, folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin are recognized as distinct variants of mycosis fungoides, because of their clinical and histological features, behavior, and /or prognosis. Mycosis fungoides often shows diagnostic difficulties, due to its absence of specific features and lesional polymorphism. A patient's treatment requires staging. In about 10% of cases, mycosis fungoides can progress to lymph nodes and internal organs. Prognosis is poor at advanced stage and management needs a multidisciplinary team approach. Advanced stage disease including tumors, erythroderma, and nodal, visceral, or blood involvement needs skin directed therapy associated with systemic drugs. Skin directed therapy includes steroids, nitrogen mustard, bexarotene gel, phototherapy UVB, and photochemiotherapy, i.e., total skin electron radiotherapy. Systemic therapies include retinoids, bexarotene, interferon, histone deacetylase inhibitors, photopheresis, targeted immunotherapy, and cytotoxic chemotherapy. Complexity of mycosis fungoides associated with long-term chronic evolution and multiple therapy based on disease stage need a multidisciplinary team approach to be treated.
RESUMO
Topical photodynamic therapy (PDT) is a non-invasive treatment modality frequently used in dermatology to treat superficial skin cancers but also some inflammatory or infectious dermatoses. PDT appears a more and more promising therapeutic option also for cutaneous lymphomas, either of T- or B-cell origin. It is a well-tolerated treatment and has excellent cosmetic outcomes, less side effects compared to other therapies (steroids, surgery, radiotherapy, and so on), no particular contraindications, and is easily repeatable in case of relapses. However, how PDT works in the treatment of cutaneous lymphoproliferative diseases is poorly understood and the literature data are still controversial. Further randomized, controlled clinical trials involving a greater number of patients and centers with a long follow-up are necessary to assess the efficacy of PDT and establish a unique standardized treatment protocol in relation to the lymphomatous disease and the type, thickness, and location of the lesions.