Mycosis fungoides and Sézary syndrome: clinical presentation, diagnosis, staging, and therapeutic management

Abstract

Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS.

Sezary Syndrome: A Case Report and Review of Current Therapeutics

“…Mycosis fungoides (MF) is the most common type of CTCL accounting for approximately 57% of CTCLs [ 1 ]. MF presents with localized or widespread skin lesions (patch, plaque, or tumor) and in some cases, lymph nodes, blood, and viscera may also be involved [ 2 ]. Sezary syndrome (SS) is a rare type of CTCL characterized by erythroderma, generalized lymphadenopathy, and clonally related malignant T-cells in the skin and peripheral blood [ 2 ].…”

“…MF presents with localized or widespread skin lesions (patch, plaque, or tumor) and in some cases, lymph nodes, blood, and viscera may also be involved [ 2 ]. Sezary syndrome (SS) is a rare type of CTCL characterized by erythroderma, generalized lymphadenopathy, and clonally related malignant T-cells in the skin and peripheral blood [ 2 ]. The incidence of SS/MF increases with age with the majority of cases over 40 years of age [ 1 , 2 ].…”

“…Sezary syndrome (SS) is a rare type of CTCL characterized by erythroderma, generalized lymphadenopathy, and clonally related malignant T-cells in the skin and peripheral blood [ 2 ]. The incidence of SS/MF increases with age with the majority of cases over 40 years of age [ 1 , 2 ]. In the United States, the incidence is higher in men, non-Hispanic black patients, individuals in high socioeconomic status quintiles, and people living in metropolitan counties [ 1 ].…”

A Narrative Review of the State of the Art of CCR4-Based Therapies in Cutaneous T-Cell Lymphomas: Focus on Mogamulizumab and Future Treatments

“…MF is characterised by indolent behaviour in the early stages, while advanced phases are regarded as an aggressive disease like SS [ 5 , 6 , 7 , 8 , 9 ]. Based on such clinical behaviour, it is unsurprising that skin-directed therapies are recommended in early MF, while systemic drugs are warranted in SS and advanced MF [ 10 ].…”

Mycosis Fungoides: A Necessary Differential Diagnosis in Infectious Disease and Dermatology Settings

“…MF significantly resembles various benign inflammatory skin conditions 3 . However, delayed diagnosis, as seen here, amplifies the likelihood of unfavorable outcomes 4 .…”