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青春期前发病的外阴硬化性苔藓:维持治疗对长期预后的重要性。

Prepubertal-Onset Vulvar Lichen Sclerosus: The Importance of Maintenance Therapy in Long-Term Outcomes.

作者信息

Ellis Elizabeth, Fischer Gayle

机构信息

Department of Dermatology, Women's and Children's Hospital, North Adelaide, South Australia, Australia.

Department of Dermatology, University of Sydney, Sydney, New South Wales, Australia.

出版信息

Pediatr Dermatol. 2015 Jul-Aug;32(4):461-7. doi: 10.1111/pde.12597. Epub 2015 May 7.

DOI:10.1111/pde.12597
PMID:25950247
Abstract

BACKGROUND

There is sufficient published data on induction treatment with potent topical corticosteroid (TCS) in childhood vulvar lichen sclerosus (VLS) but limited data on long-term management. VLS has been shown to extend beyond menarche and repercussions of suboptimal long-term control may be devastating and permanent. This single-center retrospective study reviewed outcomes of long-term treatment using individualized regimens with target outcome of complete objective normality.

METHODS

Forty-six girls with prepubertal-onset VLS were studied for demographic data, previous treatment, induction and maintenance treatment, clinical response and compliance. Photographic records were available for all patients. The cohort was divided into two groups: adherent (using treatment all or most of the time) and non-adherent (using treatment some or less of the time).

RESULTS

Twenty-six patients (56%) had received prior treatment however only three had achieved disease control using daily potent TCS. Twelve patients (26%) had scarring on presentation. All achieved initial objective disease suppression with induction treatment using potent TCS. Thirty-one of 33 adherent patients (93.93%) sustained complete disease remission with no progression or scarring. In contrast, 1 of the 13 nonadherent patients (8%) achieved complete disease remission (p < 0.001), 9 of the 13 (69.23%) experienced disease progression (p < 0.001) and 3 of the 13 (23%) developed scarring during follow-up. Those with established scarring on presentation did not recover with treatment.

CONCLUSION

Our data support previous studies regarding initial treatment with potent TCS but additionally suggest maintenance treatment with individualized regimens that achieve objective normality in addition to symptom control provide optimal outcomes.

摘要

背景

关于强效外用糖皮质激素(TCS)用于儿童外阴硬化性苔藓(VLS)的诱导治疗,已有足够的公开数据,但长期管理方面的数据有限。VLS已被证明会持续到初潮之后,长期控制不佳的后果可能是毁灭性的且不可逆转。这项单中心回顾性研究评估了采用个体化方案进行长期治疗的效果,目标是实现完全客观正常的结果。

方法

对46例青春期前发病的VLS女孩进行了研究,收集其人口统计学数据、既往治疗情况、诱导和维持治疗情况、临床反应及依从性。所有患者均有照片记录。该队列分为两组:依从组(全部或大部分时间使用治疗)和非依从组(部分或较少时间使用治疗)。

结果

26例患者(56%)曾接受过治疗,但仅3例通过每日使用强效TCS实现了疾病控制。12例患者(26%)就诊时已有瘢痕形成。所有患者通过使用强效TCS进行诱导治疗均实现了初始客观疾病抑制。33例依从组患者中有31例(93.93%)维持了完全疾病缓解,无病情进展或瘢痕形成。相比之下,13例非依从组患者中有1例(8%)实现了完全疾病缓解(p < 0.001),13例中有9例(69.23%)病情进展(p < 0.001),13例中有3例(23%)在随访期间出现瘢痕形成。就诊时已有既定瘢痕形成的患者经治疗未恢复。

结论

我们的数据支持先前关于强效TCS初始治疗的研究,但此外还表明,采用个体化方案进行维持治疗,除了控制症状外还能实现客观正常,可提供最佳结果。

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