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Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome

Primary cutaneous lymphomas represent a diverse spectrum of T-cell and B-cell lymphomas with their primary skin manifestation. Among these, mycosis fungoides (MF) and Sézary syndrome (SS) represent classic forms of cutaneous T-cell lymphomas (CTCLs). This report details the case of a 67-year-old fem...

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Autores principales: Abreu Rocha, Cláudia, Drummond Borges, Margarida, Santos, Guida Maria, Sousa, Miriam, Teixeira, Tânia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10621883/
https://www.ncbi.nlm.nih.gov/pubmed/37927614
http://dx.doi.org/10.7759/cureus.46427
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author Abreu Rocha, Cláudia
Drummond Borges, Margarida
Santos, Guida Maria
Sousa, Miriam
Teixeira, Tânia
author_facet Abreu Rocha, Cláudia
Drummond Borges, Margarida
Santos, Guida Maria
Sousa, Miriam
Teixeira, Tânia
author_sort Abreu Rocha, Cláudia
collection PubMed
description Primary cutaneous lymphomas represent a diverse spectrum of T-cell and B-cell lymphomas with their primary skin manifestation. Among these, mycosis fungoides (MF) and Sézary syndrome (SS) represent classic forms of cutaneous T-cell lymphomas (CTCLs). This report details the case of a 67-year-old female who presented with longstanding pruritic skin lesions, initially misdiagnosed and managed as eczema. The diagnostic process ultimately revealed the presence of Sézary cells in the peripheral blood smear (PBS). The SS diagnosis was confirmed based on CD4 positivity and CD7 negativity as determined by flow cytometry. The disease was staged as IVA1 (T2N0M1B2). The patient exhibited partial improvement with oral corticosteroid therapy. This report underscores the critical importance of integrating clinical evaluation and blood findings to distinguish between MF and SS. The progression of a circulating clone signals a poor prognosis, requiring surveillance and consideration of targeted therapies to enhance patient outcomes and improve their quality of life. Early detection remains paramount in the management of these rare cutaneous lymphomas, which are associated with unique therapeutic challenges.
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spelling pubmed-106218832023-11-03 Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome Abreu Rocha, Cláudia Drummond Borges, Margarida Santos, Guida Maria Sousa, Miriam Teixeira, Tânia Cureus Family/General Practice Primary cutaneous lymphomas represent a diverse spectrum of T-cell and B-cell lymphomas with their primary skin manifestation. Among these, mycosis fungoides (MF) and Sézary syndrome (SS) represent classic forms of cutaneous T-cell lymphomas (CTCLs). This report details the case of a 67-year-old female who presented with longstanding pruritic skin lesions, initially misdiagnosed and managed as eczema. The diagnostic process ultimately revealed the presence of Sézary cells in the peripheral blood smear (PBS). The SS diagnosis was confirmed based on CD4 positivity and CD7 negativity as determined by flow cytometry. The disease was staged as IVA1 (T2N0M1B2). The patient exhibited partial improvement with oral corticosteroid therapy. This report underscores the critical importance of integrating clinical evaluation and blood findings to distinguish between MF and SS. The progression of a circulating clone signals a poor prognosis, requiring surveillance and consideration of targeted therapies to enhance patient outcomes and improve their quality of life. Early detection remains paramount in the management of these rare cutaneous lymphomas, which are associated with unique therapeutic challenges. Cureus 2023-10-03 /pmc/articles/PMC10621883/ /pubmed/37927614 http://dx.doi.org/10.7759/cureus.46427 Text en Copyright © 2023, Abreu Rocha et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Family/General Practice
Abreu Rocha, Cláudia
Drummond Borges, Margarida
Santos, Guida Maria
Sousa, Miriam
Teixeira, Tânia
Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome
title Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome
title_full Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome
title_fullStr Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome
title_full_unstemmed Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome
title_short Itchy Skin: A Challenging Differential Diagnosis Between Mycosis Fungoides and Sézary Syndrome
title_sort itchy skin: a challenging differential diagnosis between mycosis fungoides and sézary syndrome
topic Family/General Practice
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10621883/
https://www.ncbi.nlm.nih.gov/pubmed/37927614
http://dx.doi.org/10.7759/cureus.46427
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