鱼鳞病治疗回顾与最新进展
鱼鳞病是一种由角质细胞分化和表皮屏障功能异常的皮肤疾病，在临床上以全身皮肤鳞屑为特点。鱼鳞病根据发病原因分为获得性鱼鳞病及遗传性鱼鳞病，其中以遗传性鱼鳞病较为常见，其遗传模式多样，包括常染色体显性遗传、常染色体隐性遗传和X染色体-连锁遗传方式。
鱼鳞病的治疗相对较困难，鱼鳞病的治疗一直是一个医学难题，现有的治疗方法和手段效果欠佳，部分治疗可能效果尚可，但是副作用较大。著名的皮肤科杂志J Am Acad Dermatol （JAAD）发表一文，介绍了鱼鳞病的治疗新进展。
目前鱼鳞病的治疗方法有局部治疗有系统性治疗。局部治疗多使用润肤剂、卡泊三醇软膏、利阿唑（Liarozole）乳膏等，治疗效果尚佳，且副作用较少。但是局部药物使用最大量每周不要超过100g。系统性治疗方法有口服维甲酸类药物。利阿唑是一种维A酸的模仿物，可阻断维A酸的代谢，增加血及组织中内源性维A酸来阐扬作用。可以系统性应用治疗鱼鳞病，但是其口服治疗鱼鳞病相比于维甲酸类药物并有优越性，其疗效和远期副作用有待进一步研究。
The ichthyoses comprise a group of inherited disorders of keratinization. Because of the need for lifelong treatment, it is important that therapies are beneficial, safe, and well tolerated.
We sought to review the evidence on existing treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.
We undertook a systematic review using the methodology of the Cochrane Collaboration. Articles published in MEDLINE, EMBASE, and CENTRAL and registered clinical trials were screened. Randomized controlled trials involving patients with the inherited ichthyoses, either syndromic or nonsyndromic but excluding ichthyosis vulgaris, were considered.
Six trials met the inclusion criteria. Topical treatments including 5% urea, 20% propylene glycol alone or in combination with 5% lactic acid, calcipotriol ointment, and liarozole 5% cream showed therapeutic benefit. Oral liarozole, a retinoic acid metabolism blocking agent, showed no advantage over oral acitretin.
Most studies were performed on a small sample of patients and lacked methodological and reporting quality. The small number oftrials and the nearly constant positive results make publication bias likely. The absence of standardization of outcome measures precluded the comparison of studies.
Topical treatments including emollients, calcipotriol ointment, and liarozole cream seem to have therapeutic benefit and a good safety profile, although the use of topical calcipotriol is limited by a maximum weekly dose of 100 g. The advantage of oral liarozole over acitretin is uncertain. Multicenter trials comparing oral and topical interventions and evaluation of long-term outcomes are needed.
参考文献：Hernández-Martin A et.al. A systematic review of clinical trials of treatments for the congenital ichthyoses, excluding ichthyosis vulgaris. J Am Acad Dermatol. 2013 Jul 16.
鱼鳞病的治疗相对较困难，鱼鳞病的治疗一直是一个医学难题，现有的治疗方法和手段效果欠佳，部分治疗可能效果尚可，但是副作用较大。著名的皮肤科杂志J Am Acad Dermatol （JAAD）发表一文，介绍了鱼鳞病的治疗新进展。
目前鱼鳞病的治疗方法有局部治疗有系统性治疗。局部治疗多使用润肤剂、卡泊三醇软膏、利阿唑（Liarozole）乳膏等，治疗效果尚佳，且副作用较少。但是局部药物使用最大量每周不要超过100g。系统性治疗方法有口服维甲酸类药物。利阿唑是一种维A酸的模仿物，可阻断维A酸的代谢，增加血及组织中内源性维A酸来阐扬作用。可以系统性应用治疗鱼鳞病，但是其口服治疗鱼鳞病相比于维甲酸类药物并有优越性，其疗效和远期副作用有待进一步研究。
BACKGROUND:
The ichthyoses comprise a group of inherited disorders of keratinization. Because of the need for lifelong treatment, it is important that therapies are beneficial, safe, and well tolerated.
OBJECTIVES:
We sought to review the evidence on existing treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.
METHOD:
We undertook a systematic review using the methodology of the Cochrane Collaboration. Articles published in MEDLINE, EMBASE, and CENTRAL and registered clinical trials were screened. Randomized controlled trials involving patients with the inherited ichthyoses, either syndromic or nonsyndromic but excluding ichthyosis vulgaris, were considered.
RESULTS:
Six trials met the inclusion criteria. Topical treatments including 5% urea, 20% propylene glycol alone or in combination with 5% lactic acid, calcipotriol ointment, and liarozole 5% cream showed therapeutic benefit. Oral liarozole, a retinoic acid metabolism blocking agent, showed no advantage over oral acitretin.
LIMITATIONS:
Most studies were performed on a small sample of patients and lacked methodological and reporting quality. The small number oftrials and the nearly constant positive results make publication bias likely. The absence of standardization of outcome measures precluded the comparison of studies.
CONCLUSIONS:
Topical treatments including emollients, calcipotriol ointment, and liarozole cream seem to have therapeutic benefit and a good safety profile, although the use of topical calcipotriol is limited by a maximum weekly dose of 100 g. The advantage of oral liarozole over acitretin is uncertain. Multicenter trials comparing oral and topical interventions and evaluation of long-term outcomes are needed.
参考文献：Hernández-Martin A et.al. A systematic review of clinical trials of treatments for the congenital ichthyoses, excluding ichthyosis vulgaris. J Am Acad Dermatol. 2013 Jul 16.
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