Cargando…

Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides

Hypopigmented mycosis fungoides is a rare form of mycosis fungoides that is characterized by achromic lesions, early onset of disease, a predilection for darker skinned populations, and a predominance of CD8+ T cells. Due to the rarity and heterogeneous presentation of hypopigmented mycosis fungoide...

Descripción completa

Detalles Bibliográficos
Autores principales: HU, Zhong-Hui, LU, Lu, FENG, Jin-Di, SONG, Hong-Bin, ZHANG, Shi-Yu, YANG, Lu, WANG, Tao, LIU, Yue-Hua
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Medical Journals Sweden, on behalf of the Society for Publication of Acta Dermato-Venereologica 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10461309/
https://www.ncbi.nlm.nih.gov/pubmed/37606155
http://dx.doi.org/10.2340/actadv.v103.6226
_version_ 1785097822820892672
author HU, Zhong-Hui
LU, Lu
FENG, Jin-Di
SONG, Hong-Bin
ZHANG, Shi-Yu
YANG, Lu
WANG, Tao
LIU, Yue-Hua
author_facet HU, Zhong-Hui
LU, Lu
FENG, Jin-Di
SONG, Hong-Bin
ZHANG, Shi-Yu
YANG, Lu
WANG, Tao
LIU, Yue-Hua
author_sort HU, Zhong-Hui
collection PubMed
description Hypopigmented mycosis fungoides is a rare form of mycosis fungoides that is characterized by achromic lesions, early onset of disease, a predilection for darker skinned populations, and a predominance of CD8+ T cells. Due to the rarity and heterogeneous presentation of hypopigmented mycosis fungoides, there are no criteria that clearly define the clinical characteristics and treatment regimens for this condition. This retrospective study of 44 paediatric patients with hypopigmented mycosis fungoides aimed to summarize their epidemiological and clinical characteristics and assess the effectiveness and safety of different treatment regimens. Clinical manifestations were further classified into 3 morphological groups: hypopigmented lesions, papules overlying hypopigmented lesions, and erythematous plaques overlying hypopigmented lesions. In addition, the results of this study suggest that interferon alpha might be an effective and well-tolerated therapy that could shorten the treatment time to complete response compared with other treatments. Maintenance therapy and long-term follow-up reduced the recurrence rate. SIGNIFICANCE Hypopigmented mycosis fungoides is an uncommon clinical variant of mycosis fungoides with heterogeneous presentations. The clinical characteristics and treatments of this rare condition have not been clarified. This study retrospectively analysed the clinical characteristics, management, and outcomes of 44 paediatric patients with hypopigmented mycosis fungoides. The clinical manifestations were categorized into 3 morphological groups: hypopigmented lesions, papules overlying hypopigmented lesions, and erythematous plaques overlying hypopigmented lesions. Furthermore, the results of this study suggest that interferon alpha might be an effective treatment that shortens the treatment time to complete response. This study provides additional information about this malignant neoplasm.
format Online
Article
Text
id pubmed-10461309
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Medical Journals Sweden, on behalf of the Society for Publication of Acta Dermato-Venereologica
record_format MEDLINE/PubMed
spelling pubmed-104613092023-08-29 Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides HU, Zhong-Hui LU, Lu FENG, Jin-Di SONG, Hong-Bin ZHANG, Shi-Yu YANG, Lu WANG, Tao LIU, Yue-Hua Acta Derm Venereol Original Article Hypopigmented mycosis fungoides is a rare form of mycosis fungoides that is characterized by achromic lesions, early onset of disease, a predilection for darker skinned populations, and a predominance of CD8+ T cells. Due to the rarity and heterogeneous presentation of hypopigmented mycosis fungoides, there are no criteria that clearly define the clinical characteristics and treatment regimens for this condition. This retrospective study of 44 paediatric patients with hypopigmented mycosis fungoides aimed to summarize their epidemiological and clinical characteristics and assess the effectiveness and safety of different treatment regimens. Clinical manifestations were further classified into 3 morphological groups: hypopigmented lesions, papules overlying hypopigmented lesions, and erythematous plaques overlying hypopigmented lesions. In addition, the results of this study suggest that interferon alpha might be an effective and well-tolerated therapy that could shorten the treatment time to complete response compared with other treatments. Maintenance therapy and long-term follow-up reduced the recurrence rate. SIGNIFICANCE Hypopigmented mycosis fungoides is an uncommon clinical variant of mycosis fungoides with heterogeneous presentations. The clinical characteristics and treatments of this rare condition have not been clarified. This study retrospectively analysed the clinical characteristics, management, and outcomes of 44 paediatric patients with hypopigmented mycosis fungoides. The clinical manifestations were categorized into 3 morphological groups: hypopigmented lesions, papules overlying hypopigmented lesions, and erythematous plaques overlying hypopigmented lesions. Furthermore, the results of this study suggest that interferon alpha might be an effective treatment that shortens the treatment time to complete response. This study provides additional information about this malignant neoplasm. Medical Journals Sweden, on behalf of the Society for Publication of Acta Dermato-Venereologica 2023-08-22 /pmc/articles/PMC10461309/ /pubmed/37606155 http://dx.doi.org/10.2340/actadv.v103.6226 Text en © 2023 Acta Dermato-Venereologica https://creativecommons.org/licenses/by-nc/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License
spellingShingle Original Article
HU, Zhong-Hui
LU, Lu
FENG, Jin-Di
SONG, Hong-Bin
ZHANG, Shi-Yu
YANG, Lu
WANG, Tao
LIU, Yue-Hua
Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides
title Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides
title_full Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides
title_fullStr Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides
title_full_unstemmed Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides
title_short Real-World Clinical Characteristics, Management, and Outcomes of 44 Paediatric Patients with Hypopigmented Mycosis Fungoides
title_sort real-world clinical characteristics, management, and outcomes of 44 paediatric patients with hypopigmented mycosis fungoides
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10461309/
https://www.ncbi.nlm.nih.gov/pubmed/37606155
http://dx.doi.org/10.2340/actadv.v103.6226
work_keys_str_mv AT huzhonghui realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides
AT lulu realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides
AT fengjindi realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides
AT songhongbin realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides
AT zhangshiyu realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides
AT yanglu realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides
AT wangtao realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides
AT liuyuehua realworldclinicalcharacteristicsmanagementandoutcomesof44paediatricpatientswithhypopigmentedmycosisfungoides