Genetics of Sjogren-Larsson Syndrome Medication

Updated: Dec 17, 2014
  • Author: William B Rizzo, MD; Chief Editor: Luis O Rohena, MD, FAAP, FACMG  more...
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Medication

Retinoids

Class Summary

These drugs are used to treat ichthyosis in patients with SLS; however, the US Food and Drug Administration (FDA) has not specifically approved retinoids for this indication. Ichthyosis has historically responded to systemic etretinate, but this long-used retinoid is no longer available. Acitretin (Soriatane) has been used off label to treat various forms of ichthyosis, but the drug is FDA approved only for the treatment of severe psoriasis in adults. The safety and efficacy of acitretin in children with Sjögren-Larsson syndrome (SLS) is not established. The prescribing physician should contact Roche Pharmaceuticals for the latest information before administering acitretin.

Acitretin (Soriatane)

Metabolite of etretinate and related to retinoic acid and retinol (vitamin A). Mechanism of action unknown, but thought to exert therapeutic effect by modulating keratinocyte differentiation, keratinocyte hyperproliferation, and tissue infiltration by inflammatory cells. Consider only in patients with severe ichthyosis that does not respond adequately to safer topical agents, such as keratolytic agents and moisturizing lotions.

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Leukotriene inhibitors

Class Summary

Inhibitors of leukotriene B4 synthesis (eg, zileuton) lower levels of this inflammatory mediator in SLS. Anecdotal experience and a small clinical trial in 5 patients with SLS suggest that zileuton therapy improves pruritus in some patients. [31] Zileuton is currently FDA approved for use in asthma. It is not approved for use in SLS or in children and, therefore, must be prescribed off label.

Zileuton (Zyflo)

Inhibits leukotriene formation, which decreases neutrophil and eosinophil migration, neutrophil and monocyte aggregation, leukocyte adhesion, capillary permeability, and smooth muscle contractions. Consider use in patients with agonizing pruritus or severe excoriations. Not all patients respond; those who do usually improve during first week of therapy.

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