Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides

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 classific...

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Main Authors: Paola Vitiello, Caterina Sagnelli, Andrea Ronchi, Renato Franco, Stefano Caccavale, Maria Mottola, Francesco Pastore, Giuseppe Argenziano, Massimiliano Creta, Armando Calogero, Alfonso Fiorelli, Beniamino Casale, Antonello Sica
Format: Article
Language:English
Published: MDPI AG 2023-02-01
Series:Healthcare
Subjects:
Online Access:https://www.mdpi.com/2227-9032/11/4/614
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author Paola Vitiello
Caterina Sagnelli
Andrea Ronchi
Renato Franco
Stefano Caccavale
Maria Mottola
Francesco Pastore
Giuseppe Argenziano
Massimiliano Creta
Armando Calogero
Alfonso Fiorelli
Beniamino Casale
Antonello Sica
author_facet Paola Vitiello
Caterina Sagnelli
Andrea Ronchi
Renato Franco
Stefano Caccavale
Maria Mottola
Francesco Pastore
Giuseppe Argenziano
Massimiliano Creta
Armando Calogero
Alfonso Fiorelli
Beniamino Casale
Antonello Sica
author_sort Paola Vitiello
collection DOAJ
description 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.
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spelling doaj.art-c11416dab35149a88251dc55c02f1ef92023-11-16T20:47:40ZengMDPI AGHealthcare2227-90322023-02-0111461410.3390/healthcare11040614Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis FungoidesPaola Vitiello0Caterina Sagnelli1Andrea Ronchi2Renato Franco3Stefano Caccavale4Maria Mottola5Francesco Pastore6Giuseppe Argenziano7Massimiliano Creta8Armando Calogero9Alfonso Fiorelli10Beniamino Casale11Antonello Sica12Dermatology Unit, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyDepartment of Mental Health and Public Medicine, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyPathology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyPathology Unit, Department of Mental and Physical Health and Preventive Medicine, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyDermatology Unit, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyDepartment of Heart Surgery and Transplantations, AORN Dei Colli-V Monaldi, 80131 Naples, ItalyRadiotherapy Unit, Emicenter, 80020 Naples, ItalyDermatology Unit, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyDepartment of Neurosciences, Reproductive Sciences and Odontostomatology, University of Naples Federico II, 80131 Naples, ItalyDepartment of Advanced Biomedical Sciences, University of Naples Federico II, 80131 Naples, ItalyThoracic Surgery Unit, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyDepartment of Pneumology and Tisiology, AO Dei Colli-V. Monaldi, 80131 Naples, ItalyDepartment of Precision Medicine, University of Campania Luigi Vanvitelli, 80131 Naples, ItalyMycosis 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.https://www.mdpi.com/2227-9032/11/4/614CTCL1primary cutaneous T-cell lymphomamycosis fungoidesmultidisciplinary team approachearly mycosis fungoides
spellingShingle Paola Vitiello
Caterina Sagnelli
Andrea Ronchi
Renato Franco
Stefano Caccavale
Maria Mottola
Francesco Pastore
Giuseppe Argenziano
Massimiliano Creta
Armando Calogero
Alfonso Fiorelli
Beniamino Casale
Antonello Sica
Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides
Healthcare
CTCL1
primary cutaneous T-cell lymphoma
mycosis fungoides
multidisciplinary team approach
early mycosis fungoides
title Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides
title_full Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides
title_fullStr Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides
title_full_unstemmed Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides
title_short Multidisciplinary Approach to the Diagnosis and Therapy of Mycosis Fungoides
title_sort multidisciplinary approach to the diagnosis and therapy of mycosis fungoides
topic CTCL1
primary cutaneous T-cell lymphoma
mycosis fungoides
multidisciplinary team approach
early mycosis fungoides
url https://www.mdpi.com/2227-9032/11/4/614
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