dermatologic disorders
Ichthyosis
Treatment of Ichthyosis
Minimization of exacerbating factors Moisturization and keratolytics Sometimes infection prophylaxis When ichthyosis is caused by a systemic disorder, the underlying disorder must be treated for the ichthyosis to abate. Other treatments of ichthyosis include emollients and keratolytics and avoiding drying ( Treatment references ). In any ichthyosis, there is impaired epidermal barrier function, and Moisturizing agents should be applied immediately after bathing. Substances that are applied to the skin may have increased absorption. For example, hexachlorophene products should not be used because of increased absorption and toxicity. An emollient, preferably plain petrolatum,mineral oil, or lotions containingureaor alpha-hydroxy acids (eg, lactic, glycolic, and pyruvic acids), should be applied twice daily, especially after bathing while the skin is still wet. Blotting with a towel removes excess applied material. Ichthyosis typically responds well to the topical keratolytic propylene glycol. To remove scale (eg, if ichthyosis is severe), patients can apply a preparation containing 40 to 60% propylene glycol in water under occlusion (eg, a thin plastic film or bag worn overnight) every night after hydrating the skin (eg, by bathing or showering); in children, the preparation should be applied twice daily without occlusion. After scale has decreased, less frequent application is required. Other useful topical agents includeurea(< 5%), dexpanthenol, ceramide-based creams, 6%salicylic acidgel, and hydrophilic petrolatum and water (in equal parts). Topical calcipotriol cream has been used with success; however, thisvitamin Dderivative can result in hypercalcemia when used over broad areas, especially in small children. Retinoidsare effective in treating inherited ichthyoses. Oral synthetic retinoids are effective for most ichthyoses.Acitretinis effective in treating most forms of inherited ichthyosis. In lamellar ichthyosis, 0.1%tretinoincream or oralisotretinoinmay be effective. The lowest effective dose should be used. Long-term (1 year) treatment with oralisotretinoinhas resulted in bony exostoses in some patients, and other long-term adverse effects may arise. Research is ongoing in the development of molecular treatments for ichthyoses and related diseases, including protein and enzyme replacement, repurposed biologic agents, small-molecule agents, and gene replacement and editing ( Treatment references ). Oral retinoids are contraindicated in pregnancy because of their teratogenicity, andacitretinshould be avoided in women of childbearing potential because of its teratogenicity and long duration of action. Patients with epidermolytic hyperkeratosis may need treatment with cloxacillin 250 mg orally 3 or 4 times a day orerythromycin250 mg orally 3 or 4 times a day for as long as thick intertriginous scale is present to prevent bacterial superinfection from causing painful, foul-smelling pustules. Regularly using soaps containingchlorhexidinemay also reduce the bacteria, but these soaps tend to dry the skin. 1.Mazereeuw-Hautier J, Vahlquist A, Traupe H, et al: Management of congenital ichthyoses: European guidelines of care, part one.Br J Dermatol180(2):272–281, 2019. doi: 10.1111/bjd.17203 2.Joosten MDW, Clabbers JMK, Jonca N, et al: New developments in the molecular treatment of ichthyosis: Review of the literature.Orphanet J Rare Dis17(1):269, 2022. doi: 10.1186/s13023-022-02430-6 