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家族性额部纤维性秃发病例系列及文献综述

Case series of familial frontal fibrosing alopecia and a review of the literature.

作者信息

Navarro-Belmonte María Rogelia, Navarro-López Vicente, Ramírez-Boscà Ana, Martínez-Andrés Ma Asunción, Molina-Gil Consuelo, González-Nebreda Miguel, Asín-Llorca Manuel

机构信息

Unit of Alopecia, Centro Dermatológico Estético de Alicante, Alicante, Spain; Department of Dermatology, Centro Dermatológico Estético de Alicante, Alicante, Spain.

出版信息

J Cosmet Dermatol. 2015 Mar;14(1):64-9. doi: 10.1111/jocd.12125. Epub 2015 Jan 23.

DOI:10.1111/jocd.12125
PMID:25614294
Abstract

Frontal fibrosing alopecia (FFA) is a distinctive form of scarring alopecia presenting with partial eyebrow loss and frontal temporal parietal recession of the hairline. Its etiology remains unknown, and there is no definitive treatment. Information in familial cases of FFA is scarce. We conducted a retrospective cohort study describing the mean clinical findings, treatment, and also the mean differences between premenopausal and postmenopausal cases of familiar FFA. Data analysis from case was performed on eight patients with a familiar history and diagnosis of FFA seen at the Alicante Aesthetic Dermatology Centre between January 2009 and June 2014. All patients in this cohort were females. Mean age at onset was 65 year (range 60-75) in the postmenopausal patients and 39 year (range 33-47) in the premenopausal women. All menopausal patients were in an advanced stage when the disease had already developed in the frontal and/or temporal parietal hairline region. However, the daughters, all of them premenopausal age, attended the consultation with mild involvement of the eyebrows in all four cases and mild impairment of the frontal hairline in three of them. Specific clinical findings in familial FFA are poorly communicated until nowadays although the number of familial cases arises until 8% in the main case series published in recent years. Early diagnosis in premenopausal stage is frequent in our case series and allows us to begin the protocol treatment in the first stage of the disease, but long-term progression will remain uncertain until a definitive treatment could be established by multicenter randomized controlled trials.

摘要

额部纤维性秃发(FFA)是瘢痕性秃发的一种特殊形式,表现为部分眉毛缺失以及发际线的额颞顶部后移。其病因尚不清楚,且没有确切的治疗方法。关于FFA家族性病例的信息很少。我们进行了一项回顾性队列研究,描述了家族性FFA绝经前和绝经后病例的平均临床特征、治疗情况以及平均差异。对2009年1月至2014年6月在阿利坎特美容皮肤科中心就诊的8例有家族病史且诊断为FFA的患者进行了病例数据分析。该队列中的所有患者均为女性。绝经后患者的平均发病年龄为65岁(范围60 - 75岁),绝经前女性为39岁(范围33 - 47岁)。所有绝经患者就诊时病情已发展至晚期,额部和/或颞顶部发际线区域已出现病变。然而,女儿们均处于绝经前年龄,4例患者均表现为眉毛轻度受累,其中3例患者额部发际线有轻度损害。尽管在近年来发表的主要病例系列中,家族性病例的比例高达8%,但至今家族性FFA的具体临床特征仍鲜有报道。在我们的病例系列中,绝经前阶段的早期诊断较为常见,这使我们能够在疾病的第一阶段开始规范治疗,但在多中心随机对照试验确定确切治疗方法之前,长期病程仍不确定。

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