Acitretin is the most studied and widely used oral retinoid for inherited disorders of keratinization. Its major disadvantage is the need for contraceptive measures three years after discontinuation. An alternative is needed for women in childbearing age. With alitretinoin, pregnancy is safe one month after discontinuation. The purpose of this study is to share our experience with alitretinoin as an alternative to acitretin for inherited disorders of keratinization in women in childbearing age, and give an overview of the literature. We included five patients, three autosomal recessive congenital ichthyosis (ARCI), one erythrokeratoderma variabilis (EKV), and one epidermolytic epidermal nevus (EEN), treated with alitretinoin 30 mg during 5-17 months. Efficacy was measured by Ichthyosis Area Severity Index (IASI) and Investigator Global Assessment (IGA). IASI was significantly reduced with a mean value of 31% and IASI-Scaling subscore of 38% (p<0,01). IGA was reduced by one point in 4/5 patients. Three patients (2 ARCI, 1 EEN) are still being treated, one patient (EKV) stopped to become pregnant, one patient (ARCI) discontinued due to financial reasons. Literature was searched in Pubmed with the Mesh terms “alitretinoin”, “skin diseases, genetic”, “ichthyosis” and “keratosis”. Results consisted of case reports and case series of alitretinoin in Darier disease, Hailey-Hailey disease, EKV, palmoplantar keratoderma, ichthyosis and ichthyosis syndromes. The vast majority reported significant improvement or even complete remission of skin symptoms. Side effects were relatively mild, except for a case of benign intracranial hypertension. However, validated outcome measures to support the results were mostly lacking. Our study uses such outcome measures and shows that alitretinoin is effective to mitigate the symptoms of the inherited keratinization disorders in women in childbearing age and a suitable alternative to acitretin.

ESDR185 - Alitretinoin as an alternative treatment for inherited diseases of keratinization in childbearing age women – a case series and literature review

50th Annual Meeting of the European Society for Dermatological Research. VIRTUAL MEETING