中华皮肤科杂志

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阿维A治疗儿童及青少年遗传角化性皮肤病的临床观察

张锡宝1, 何玉清1, 蔡艳霞1, 罗权1, 王艳芳1, 徐晓1, 李常兴2, 黄振明1, 罗玉武1, 李季1, 汤少开1   

  1. 1. 广州市皮肤病防治所 510095;
    2. 东莞市慢性病防治院皮肤性病科
  • 收稿日期:2006-02-13 出版日期:2006-04-15 发布日期:2006-04-15
  • 基金资助:
    广东省自然科学基金资助项目(04004285)

A clinical evaluation of acitretin therapy in children and adolescents with inherited keratodermas

ZHANG Xi-bao1, HE Yu-qing1, CAI Yan-xia1, LUO Quan1, WANG Yan-fang1, XU Xiao1, LI Chang-xing2, HUANG Zhen-ming1, LUO Yu-wu1, LI Ji1, TANG Shao-kai1   

  1. Guangzhou Institute of Dermatology, Guangzhou 510095, China
  • Received:2006-02-13 Online:2006-04-15 Published:2006-04-15

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摘要： 目的 观察阿维A治疗难治和泛发遗传角化性皮肤病的临床疗效、不良反应及对儿童生长发育的影响,评估阿维A在儿童患者中应用的安全性.方法 对23例难治和泛发角化性皮肤病患者分年龄段进行治疗与观察,阿维A治疗剂量为0.67-1.07mg/(kg·d),维持量为0.08-0.94mg/(kg·d).选择身高与体质量作为儿童生长发育的指标,每1-3个月随访1次,共随访6-35个月.结果 经1-6个月的治疗,临床痊愈率达82.6%,显效率为13.04%,治疗反应最好的依次是大疱性鱼鳞病、板层状鱼鳞病、毛发红糠疹、炎性线状疣状表皮痣、对称性进行性红斑角化病、先天性鱼鳞病样综合征伴耳聋及角膜炎和泛发性汗孔角化病;非大疱性鱼鳞病有效,豪猪状鱼鳞病的治疗反应最差.不良反应发生率为干燥性唇炎65.2%,皮肤瘙痒39.1%,皮肤脆性增加34.8%,口干30.4%,脱发、食欲下降分别为13.0%和8.7%,头疼与听力障碍均为4.3%.随访未见对儿童生长发育的影响.结论 阿维A治疗儿童难治和泛发遗传角化性皮肤病疗效满意,在观察期间对儿童生长发育未见明显影响.

关键词: 阿维A, 角化病, 痣, 治疗

Abstract: Objective To evaluate the therapeutic effects and safety of acitretin for severe inherited keratodermas in children and adolescents.Methods Acitretin was given to 23 children and adolescents with either lamellar ichthyosis,bulbous ichthyosiform erythroderma,pityriasis rubra pillars,progressive symmetrical erythrokeratoderma,keratitis ichthyosis deafness syndrome,generalized porokeratosis,inflammatory liner verrucous epidermal nevus,ichthyosis hystrix and non-bullous ichthyosiform erythroderma.The therapeutic dosage was 0.67-1.07 mg/(kg·d),and maintenance dosage 0.08-0.94 mg/(kg·d).The effects on the patients' growth and development of the drug were evaluated based on the changes of body weight and height in the children.The total follow-up period was 6-35 months in an interval of 1-3 months.Results The considerable overall improvement was achieved after 1-6 months' treatment,with an overall clinical cure rate of 82.6%.Only one case responded poorly to the therapy.The excellent responses were observed in patients with bulbous ichthyosiform erythroderma,lamellar ichthyosis,and pityriasis rubra pillars,etc,and the much poor responses in ichthyosis hystrix.The most frequent adverse reaction was mild to moderate dry lips (65.2%),the next were pruritus(39.1%),skin fragility(34.8%),and dry mouth(30.4%).The less frequent adverse reactions were alopecia(13%),anorexia(8.7%),headache (4.3%) and hypoacusis (4.3%).No effects on the growth and development were found in those children during the follow up period.Conclusions The considerable overall improvement is achieved with the acitretin therapy for children and adolescents with inherited keratodermas,with only mild to moderate adverse reactions and no effects on the growth and development in the children.

Key words: Acitretin, Keratinizatosis, Nevus, Therapy