Vahlquist Anders, Gånemo Agneta, Virtanen Marie
Department of Medical Sciences, Section of Dermatology, University Hospital, University of Uppsala, Uppsala, Sweden.
Acta Derm Venereol. 2008;88(1):4-14. doi: 10.2340/00015555-0415.
Congenital ichthyosis is a collective name for a group of monogenetic disorders of cornification, sometimes associated with systemic symptoms. There may be an abnormal quality or quantity of scale produced, abnormal thickness of stratum corneum or abnormal keratinocyte kinetics, often associated with skin inflammation. Pruritus, skin fragility, ectropion and anhidrosis are sometimes associated with the rare types of ichthyosis. Three important mechanisms are involved in the action of topical agents used in the treatment of ichthyosis: hydration, lubrication and keratolysis. The latter effect can also be achieved with systemic retinoids. For ichthyosis with an increased tendency towards skin infections, antimicrobials are another group of widely used agents. Considering that patients with ichthyosis are potential mega-users of topical therapy, with an estimated lifetime consumption of approximately one tonne cream per capita, surprisingly few controlled trials of the various treatments have been performed. Moreover, nearly all therapeutic principles were established long before the recent increase in knowledge about the aetiology and pathophysiology of ichthyosis. This calls for new ideas and intensified efforts to develop future ichthyosis therapies.
先天性鱼鳞病是一组角化单基因疾病的统称，有时伴有全身症状。可能存在产生的鳞屑质量或数量异常、角质层厚度异常或角质形成细胞动力学异常，常伴有皮肤炎症。瘙痒、皮肤脆弱、睑外翻和无汗有时与罕见类型的鱼鳞病相关。治疗鱼鳞病所用外用药物的作用涉及三个重要机制：水合作用、润滑作用和角质松解作用。后者也可用全身性维甲酸类药物实现。对于皮肤感染倾向增加的鱼鳞病患者，抗菌药物是另一类广泛使用的药物。鉴于鱼鳞病患者是外用治疗的潜在大量使用者，估计人均终身乳膏消费量约为1吨，令人惊讶的是，针对各种治疗方法的对照试验开展得很少。此外，几乎所有治疗原则早在最近对鱼鳞病病因和病理生理学认识增加之前就已确立。这就需要新的思路并加大力度开发未来的鱼鳞病治疗方法。
