Medication Summary

Treatment of Sjögren-Larsson syndrome (SLS) is palliative. Dermatologic therapy consists of retinoids, emollients, and keratolytic agents.^[45]However, other findings give the first support to the concept that gene therapy may be a future treatment option.^{[46, 47]}Vitamin D-3 analogues are used to reduce increased epidermal turnover.^{[48, 49]} 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.^[50]Early intervention for this disabling disorder is important.^[44]

Small aldehyde-reactive molecules should be explored as novel therapeutic agents.^[51]

Class Summary

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

Acitretin (Soriatane)

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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.

Class Summary

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

Calcipotriene (Dovonex)

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

Class Summary

These agents are used to soften the affected skin.

Urea (Ureacin-40, Aquacare)

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

Mineral oil (Kondremul, Zymenol)

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

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Jacek C Szepietowski, MD, PhD Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland

Disclosure: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

Anna Zalewska, MD, PhD Professor of Dermatology and Venereology, Psychodermatology Department, Chair of Clinical Immunology and Microbiology, Medical University of Lodz, Poland

Disclosure: Nothing to disclose.

Sections Dermatologic Manifestations of Sjogren-Larsson Syndrome
Overview
Presentation
DDx
Workup
Treatment
Medication
References

Dermatologic Manifestations of Sjogren-Larsson Syndrome Medication

Medication Summary

Retinoids