Dermatologic Manifestations of Sjogren-Larsson Syndrome Medication

Updated: Apr 16, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Medication

Medication Summary

Treatment of Sjögren-Larsson syndrome (SLS) is palliative. Dermatologic therapy consists of retinoids, emollients, and keratolytic agents. [43] However, other findings give the first support to the concept that gene therapy may be a future treatment option. [44, 45] Vitamin D-3 analogues are used to reduce increased epidermal turnover. [46, 47]  Research has shown that patients with expression of the FALDH protein and some residual enzyme activity could benefit from the hypolipemic drug benzafibrate, which is a pan-agonist of all PPAR-isoforms. [48] Early intervention for this disabling disorder is important. [42]

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Retinoids

Class Summary

These drugs are used to reduce the increased epidermal turnover in patients with Sjögren-Larsson syndrome.

Acitretin (Soriatane)

Acitretin is a retinoic acid analog, like etretinate and isotretinoin. Etretinate is the main metabolite and has demonstrated clinical effects close to those seen with etretinate. Its mechanism of action is unknown but it affects keratinocytic differentiation.

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Vitamin D-3 analogues

Class Summary

Vitamin D-3 analogues are used to reduce increased epidermal turnover.

Calcipotriene (Dovonex)

Calcipotriene is a synthetic vitamin D-3 analog that regulates skin cell production and development.

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Keratolytics

Class Summary

These agents are used to soften the affected skin.

Urea (Ureacin-40, Aquacare)

Urea promotes hydration and removal of excess keratin in conditions of hyperkeratosis. It is available in 10-40% concentrations.

Mineral oil (Kondremul, Zymenol)

Mineral oil provides relief of minor skin irritations and promotes the removal of excess keratin in conditions of hyperkeratosis.

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