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.^[39]

Surgical Care

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

Consultations

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

Dermatologist
Neurologist
Pediatrician
Physiotherapist
Ophthalmologist
Orthopedist

Diet

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.^[40]

Activity

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. According to their parents, patients were reportedly content with their quality of life.^[41]

Aggressive physiotherapy and stimulation techniques for mental retardation are good suggested modes of therapy.^[42]

Long-Term Monitoring

A multidisciplinary approach to Sjögren-Larsson syndrome (SLS) is mandatory (see Consultations). Sauna treatments (90-100°C) and frequent bathing or showering are beneficial for patients with Sjögren-Larsson syndrome. Orthopedic measures, such as hamstring release and (if kyphosis is present) thoracic braces, can enable patients with Sjögren-Larsson syndrome to walk.

Medication

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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.