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儿童期发病的外阴硬化性苔藓在青春期不会消退:一项前瞻性病例系列研究。

Childhood onset vulvar lichen sclerosus does not resolve at puberty: a prospective case series.

作者信息

Smith Saxon D, Fischer Gayle

机构信息

Dermatology Department, Royal North Shore Hospital, Sydney, New South Wales 2065, Australia.

出版信息

Pediatr Dermatol. 2009 Nov-Dec;26(6):725-9. doi: 10.1111/j.1525-1470.2009.01022.x.

Abstract

When vulvar lichen sclerosus occurs in prepubertal children it is widely believed that it is likely to remit at puberty. However when it occurs in adult women it is accepted that remission is unlikely and that in addition untreated or inadequately treated disease may be complicated by significant disturbance of vulvar architecture and less commonly squamous cell carcinoma. Our database reveals 18 girls who developed lichen sclerosus prior to puberty who are now adolescents or young adults. Twelve have remained under surveillance and the other six patients have been lost to follow-up. We report a prospective series of these 12 patients. Three patients have achieved complete remission sustained for three or more years, all prior to menarche. Nine patients, or 75% of the cohort, who still had active lichen sclerosus at puberty continue to require maintenance therapy after menarche. Of the 12, six have had significant disturbance of vulvar architecture. The concept that prepubertal lichen sclerosus resolves at puberty would appear not to be true in the majority of patients. Even when diagnosed early and treated effectively, childhood onset lichen sclerosus may be complicated by distortion of vulvar architecture.

摘要

当外阴硬化性苔藓发生在青春期前儿童时,人们普遍认为它很可能在青春期缓解。然而,当它发生在成年女性时,人们公认缓解不太可能发生,而且未经治疗或治疗不充分的疾病可能会因外阴结构的严重紊乱而复杂化,较少见的情况是并发鳞状细胞癌。我们的数据库显示有18名女孩在青春期前患上了硬化性苔藓,现在她们已是青少年或年轻成年人。12名患者仍在接受监测,另外6名患者失访。我们报告这12名患者的前瞻性系列情况。3名患者已实现持续三年或更长时间的完全缓解,均在初潮前。9名患者,即该队列中的75%,在青春期时仍患有活动性硬化性苔藓,初潮后仍继续需要维持治疗。在这12名患者中,6名患者有外阴结构的严重紊乱。青春期前硬化性苔藓在青春期会消退这一概念在大多数患者中似乎并不成立。即使早期诊断并有效治疗,儿童期发病的硬化性苔藓仍可能因外阴结构扭曲而复杂化。

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