Clinical investigation of acitretin in children with severe inherited keratinization disorders in China.

Objective: Investigation into the clinical efficacy, side effects and safety of oral acitretin on severe inherited disorders of keratinization in children. Methods: Acitretin was given as a treatment dose of 0.77-1.07 mg/kg.per day (mean 0.86 +/- 0.11) and maintenance dose of 0-0.94 mg/kg.per day (mean 0.33 +/- 0.26) to 28 children with severe inherited disorders of keratinization. Body height and weight were chosen as the monitoring indexes to evaluate the growth and development and other common side effects as the safety evaluation of the children for a follow-up of 2-36 months. Results: After 2-4 months of treatment, the clinical cure rate was 82.1% and the effective rate was 17.9%. Most cases, such as bullous ichthyosiform erythroderma, lamellar ichthyosis, pityriasis rubra pilaris, and inflammatory linear verrucous epidermal nevus showed remarkable therapeutic response; non-bullous ichthyosiform erythroderma was also effective. Two cases with Darier's disease were previously shown to be resistant to acitretin therapy, but improved after 6 months of treatment. No previous investigation had been made on a negative effect on the growth and development of such children. Conclusion: Acitretin showed a satisfactory therapeutic effect on severe inherited disorders of keratinization in children.