Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, State Key Laboratory of Complex Severe and Rare Diseases, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, China.
Acta Derm Venereol. 2023 Aug 22;103:adv6226. doi: 10.2340/actadv.v103.6226.
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.
色素减退性蕈样肉芽肿是一种罕见的蕈样肉芽肿，其特征为色素减退性损害、疾病早期发病、偏爱深色皮肤人群以及以 CD8+T 细胞为主。由于色素减退性蕈样肉芽肿的罕见性和异质性表现，目前尚无明确界定该疾病临床特征和治疗方案的标准。本项回顾性研究纳入了 44 例色素减退性蕈样肉芽肿患儿，旨在总结其流行病学和临床特征，并评估不同治疗方案的有效性和安全性。临床表现进一步分为 3 种形态学组：色素减退性损害、色素减退性损害上的丘疹和色素减退性损害上的红斑性斑块。此外，本研究结果表明，与其他治疗方法相比，干扰素α可能是一种有效且耐受性良好的治疗方法，可缩短达到完全缓解的治疗时间。维持治疗和长期随访可降低复发率。
