Abstract: Objective To study the acitretin as the treatment for children cases with severe inherited disorders of keratinization.Clinical data of efficacy and tolerability of acitretin therapy were available for all patients and defines guidelines for treatment.Methods 22 children with severe inherited disorders of keratinization had been treated by acitretin,the body weight and height had been adopted to investigate the growth and development of children,the treatment and maintancedose were(0.67)～(1.07) mg/(kg·d) and 0～0.94 mg/(kg·d) respectively and the cumulative follow-up was 3～36 months for these 22 patients.Results The overall improvement was considerable;the clinical cured and effective rate were 77.3% and 18.2%,with only one case responding poorly.Most of those cases were showed the excellent response.Mild to moderate dry lips was most frequent((54.5)%),the next were pruritus(36.4%),skin fragility(27.3%),and dry mouth(22.7%).The less frequent side effects were anorexie(13.6%),and hypoacusis(4.5%).Irreversible side effects did not occur and showed no any effected on the growth and development of those children cases during treatment in the study.Conclusion The overall improvement of acitretin therapy in treatment of severe disorders of keratinization is considerable,with only mild to moderate side effects and has no effected on the growth and development in children.Acitretin therapy represents the treatment of choice for severe inherited disorders of keratinization and it is a safe and effective treatment during the investigation.

Key words: acitretin, keratinigztion disorders, treatment, children