背景:Acitretin,合成的维生素A衍生物,是研究最多和广泛使用的用于鱼鳞病的口服类维生素A。其主要缺点是停药后三年内需要采取避孕措施。育龄妇女需要一种替代方法。用阿利维甲酸,另一种类维生素A,停药一个月后怀孕被认为是安全的。
目的:为阿利维甲酸替代阿维A素治疗育龄妇女鱼鳞病提供证据。我们的经验在一个案例系列中分享,并结合了当前文献的概述。
方法:9名育龄妇女(19-31岁,中位数21)具有不同亚型的鱼鳞病(常染色体隐性遗传先天性鱼鳞病,(浅表)表皮松解性鱼鳞病,易变性红斑角化病,表皮松解性表皮痣,包括表皮性鱼鳞病的马赛克形式),并在2-28个月内用30mg阿利维甲酸治疗。严重程度通过鱼鳞病面积严重程度指数(IASI)和研究者全球评估(IGA)进行测量。使用网格术语“阿利维甲酸”在Pubmed中进行文献检索,“皮肤病,进行了遗传性“鱼鳞病”和“鱼鳞病”。
结果:IGA的平均得分显着降低,IASI-红斑,可见IASI缩放和IASI总计。七名病人仍在接受治疗,一名病人停止怀孕,一名患者因经济原因停药。观察到的副作用是可逆性头痛(n=6),脂性湿疹(n=1),\'暂时感觉不舒服\'(n=1)和脚更容易起泡(n=1)。文献检索产生了6例关于阿利维甲酸在鱼鳞病和鱼鳞病综合征中的病例报告和病例系列,共有29例患者。绝大多数(21/29)报告了皮肤症状的显着改善甚至完全缓解。然而,缺乏支持这些结果的验证结果指标.副作用(n=16)相对轻微,除了良性颅内高压(n=1)和自身免疫性甲状腺功能减退症(n=1)。
结论:我们的研究表明,通过验证的结果衡量标准,阿利维A酸可有效缓解育龄妇女鱼鳞病的症状,也是阿维A的合适替代品。
BACKGROUND: Acitretin, a synthetic vitamin A derivative, is the most studied and widely used oral retinoid for ichthyoses. Its major disadvantage is the need for contraceptive measures during 3 years after discontinuation. An alternative is needed for women of childbearing age. With
alitretinoin, another retinoid, pregnancy is considered safe 1 month after discontinuation.
OBJECTIVE: The aim of this study was to provide evidence for
alitretinoin as an alternative for acitretin for ichthyosis in women of childbearing age. Our experience is shared in a case series combined with an overview of the current literature.
METHODS: Nine women of childbearing age (19-31 years, median 21) with different subtypes of ichthyosis (autosomal recessive congenital ichthyosis, (superficial) epidermolytic ichthyosis, erythrokeratoderma variabilis, and epidermolytic epidermal nevi, a mosaic form of epidermolytic ichthyosis) were included and treated with 30 mg alitretinoin during 2-28 months. Severity was measured by Ichthyosis Area Severity Index (IASI) and Investigator Global Assessment (IGA). A literature search in Pubmed using the Mesh terms \"
alitretinoin,\" \"skin diseases, genetic\" and \"ichthyosis\" was performed.
RESULTS: Significant reduction in the mean scores of IGA, IASI-erythema, IASI-scaling, and IASI-total was seen. Seven patients are still being treated, 1 patient stopped to become pregnant, 1 patient discontinued due to financial reasons. Observed side effects were reversible headache (n = 6), asteatotic eczema (n = 1), \"not feeling well\" temporarily (n = 1), and easier blistering of the feet (n = 1). The literature search resulted in six case reports and case series about
alitretinoin in ichthyosis and ichthyosis syndromes with in total 29 patients. The vast majority of articles (21/29) reported significant improvement or even complete remission of skin symptoms. However, validated outcome measures to support these results were lacking. Side effects (n = 16) were relatively mild, except for benign intracranial hypertension (n = 1) and autoimmune hypothyroidism (n = 1).
CONCLUSIONS: Our study shows, with validated outcome measures, that
alitretinoin is effective to mitigate the symptoms of ichthyosis in women of childbearing age and a suitable alternative to acitretin.