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