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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...

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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
Descripción
Sumario: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.