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新加坡儿童蕈样肉芽肿:46例患儿系列研究

Pediatric mycosis fungoides in Singapore: a series of 46 children.

作者信息

Heng Yee Kiat, Koh Mark Jean Aan, Giam Yoke Chin, Tang Mark Boon Yang, Chong Wei Sheng, Tan Suat Hoon

机构信息

National Skin Centre, Singapore, Singapore.

出版信息

Pediatr Dermatol. 2014 Jul-Aug;31(4):477-82. doi: 10.1111/pde.12352. Epub 2014 May 29.

DOI:10.1111/pde.12352
PMID:24890628
Abstract

Few studies have evaluated Asian children with mycosis fungoides (MF). We report a series of patients from a tertiary dermatologic institution in Singapore. A retrospective review was performed of patients younger than 16 years old diagnosed with MF between 2000 and 2008 at the National Skin Centre, Singapore. Forty-six patients were identified. At initial presentation, a provisional diagnosis of MF was made in 19 patients (41.3%), pityriasis lichenoides chronica (PLC) in 11 (23.9%) and postinflammatory hypopigmentation due to eczema or other causes in 11 (23.9%). After skin biopsy, the hypopigmented variant of MF was diagnosed in 42 patients (91.3%). There was one case each of PLC-like MF, pigmented purpuric dermatosis-like MF, classic MF, and solitary MF. Pityriasis lichenoides coexisted in three cases (6.5%). All except one patient presented with the early patch-plaque stage of disease (stage IA/B). The disease did not progress in any of our patients after a mean follow-up of 71.0 ± 52.5 months. Twenty-seven patients (58.7%) had complete disease clearance after a mean duration of 27.1 ± 28.1 months; 15 (49.7%) of 32 patients who received narrowband ultraviolet B treatment had complete clearance within an average of 8.9 ± 5.3 months, but 7 patients relapsed within 14.9 ± 14.8 months. One patient with solitary MF failed multiple treatment modalities before eventually achieving disease clearance with photodynamic therapy. Hypopigmented MF is the most common MF variant in Asian children. The diagnostic difficulty is in differentiating this from PLC, which may coexist with MF. Long-term prognosis is generally favorable.

摘要

很少有研究评估亚洲蕈样肉芽肿(MF)患儿。我们报告了新加坡一家三级皮肤病机构的一系列患者。对2000年至2008年在新加坡国家皮肤中心诊断为MF的16岁以下患者进行了回顾性研究。共确定了46例患者。初诊时,19例患者(41.3%)初步诊断为MF,11例(23.9%)为慢性苔藓样糠疹(PLC),11例(23.9%)为湿疹或其他原因引起的炎症后色素减退。皮肤活检后,42例患者(91.3%)被诊断为色素减退型MF。各有1例PLC样MF、色素性紫癜性皮病样MF、经典MF和孤立性MF。3例(6.5%)患者同时存在苔藓样糠疹。除1例患者外,所有患者均处于疾病早期斑块期(IA/B期)。平均随访71.0±52.5个月后,所有患者病情均未进展。27例患者(58.7%)平均27.1±28.1个月后疾病完全清除;32例接受窄谱中波紫外线治疗的患者中有15例(49.7%)平均8.9±5.3个月内完全清除,但7例患者在14.9±14.8个月内复发。1例孤立性MF患者在多种治疗方法均失败后,最终通过光动力疗法实现疾病清除。色素减退型MF是亚洲儿童中最常见的MF亚型。诊断困难在于将其与可能与MF共存的PLC区分开来。长期预后总体良好。

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Childhood hypopigmented mycosis fungoides: a commonly delayed diagnosis.儿童色素减退性蕈样肉芽肿:一种常被延迟诊断的疾病。
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