Systemic retinoids in the management of ichthyoses and related skin types

Abstract

The term retinoid includes both natural and synthetic derivatives of vitamin A. Retinoid containing treatments have been used since the early Egyptians ~1550BC. Treatment of ichthyosiform disorders with retinoids dates back at least to the 1930’s. Early use of high dose vitamin A demonstrated efficacy but, because vitamin A is stored in the liver, toxicity limited usefulness. Interest turned to synthetic retinoids in an effort to enhance efficacy and limit toxicity. Acetretin, isotretinoin and, in the past etretinate, have provided the most effective therapy for ichthyosiform conditions. They have been used for a variety of ages, including in newborns with severe ichthyosis and for decades in some patients. Careful surveillance and management of mucous membrane, laboratory, skeletal, and teratogenic side effects has made systemic retinoids the mainstay of therapy for ichthyosis and related skin types.

Vitamin D: A New Promising Therapy for Congenital Ichthyosis

“…With retinoid therapy, the improvement in skin thickness and scaling begins in ∼1-2 weeks of starting the treatment in ARCI. 14 The response, however, is variable and does not lead to near complete clearance of scaling (personal observation), as reported in the present case series with vitamin D supplementation. In all our cases, the parents noticed reduction in stiffness within 2 to 3 days of vitamin D supplementation, indicating an immediate response.…”

Rachitisme carentiel compliquant une maladie de Chanarin-Dorfman

“…Un article récent sur les traitements topiques de l'ichtyose a insisté sur le fait qu'il n'existait pas de traitement efficace pour tous les patients car il y a une grande variabilité individuelle en fonction de l'âge et des activités [12] en dehors des traitements émollients toujours utiles. Les réti-noïdes oraux semblent efficaces dans cette indication moyennant une surveillance des effets secondaires [13,14].…”

Lamellar ichthyosis and arthrogryposis in a premature neonate

“…These include development of ligamentous calcifications, skeletal hyperostosis, liver and mucocutaneous toxicity (cheilitis, epistaxis, and eye irritation). 5 In our patient, clinical improvement was achieved only after treatment with oral acitretin and the incredibly quick resolution of such a destructive clinical picture highlights the importance of early administration of a specific therapy.…”

“…Oral retinoids are generally recommended in neonates with severe skin involvement, but not in all cases. [4][5] The benefit/risk ratio must be carefully balanced, as systemic retinoid therapy has significant short and long-term adverse effects. These include development of ligamentous calcifications, skeletal hyperostosis, liver and mucocutaneous toxicity (cheilitis, epistaxis, and eye irritation).…”