Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides

Abstract

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.

Keywords: CTCL1; early mycosis fungoides; multidisciplinary team approach; mycosis fungoides; primary cutaneous T-cell lymphoma.

Figure 1

(A) Typical clinical presentation with erythematous patches in the bathing suit area. (B) Patient presenting multiple typical erythematous plaques on the trunk. (C) Genital tumor with superficial erosions. (D) Alopecic patch on forearm in patient with folliculotropic mycosis fungoides.

Figure 2

Histological clues of MF. Lymphocytes are aligned along the dermal-epidermal junction; they are surrounded by a halo and are larger than the dermal lymphocytes ((A), H&E, original magnification 100×). The alignment of lymphocytes is highlighted by CD3 immunohistochemistry ((B), CD3 immunostain, original magnification 200×). Some intraepidermal lymphocytes show irregular and hyperchromatic nuclei (red arrow) ((C), H&E immunostain, original magnification 400×). Pautrier’s microabscesses are characterized by aggregates of atypical lymphocytes in the thickening of the epidermis, which shows no significant spongiosis. In this image, a mitosis is also evident ((D), H&E, original magnification 200×).