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Dermatologic Manifestations of Sjogren-Larsson Syndrome Treatment & Management

  • Author: Anna Zalewska, MD, PhD; Chief Editor: William D James, MD  more...
Updated: Sep 18, 2013

Medical Care

Curative Sjögren-Larsson syndrome (SLS) treatment regimens are currently not available; however, studies provide some data on leukotriene synthesis inhibitors as possible therapeutic agents for patients with Sjögren-Larsson syndrome.[27]


Surgical Care

Early physiotherapy and later soft-tissue surgery may be helpful in treating patients with Sjögren-Larsson syndrome.



Sjögren-Larsson syndrome is a multisystem disease and requires the attention of many specialists, including the following:

  • Dermatologist
  • Neurologist
  • Pediatrician
  • Physiotherapist
  • Ophthalmologist
  • Orthopedist


Dietary restrictions for patients with Sjögren-Larsson syndrome that focused on reducing intake of long-chain fatty acids were mostly ineffective; however, some authors maintain that a low-fat diet with a medium-chain fatty acid addition could lead to skin lesion improvement, especially when started early in infancy and with high compliance.[28]



The clinical spectrum of patients with Sjögren-Larsson syndrome varies from those who are wheelchair-bound to those who can walk.

  • Spasticity is usually so severe that many patients are never able to walk.
  • Later in development (around age 3-5 y), severe neurologic deterioration prevents patients with Sjögren-Larsson syndrome from walking.
Contributor Information and Disclosures

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.


Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD 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: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

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: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; 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 i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

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: Nothing to disclose.

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; .

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