Dermatologic Manifestations of Sjogren-Larsson Syndrome Treatment & Management

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

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]

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Surgical Care

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

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Consultations

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

  • Dermatologist

  • Neurologist

  • Pediatrician

  • Physiotherapist

  • Ophthalmologist

  • Orthopedist

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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]

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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]

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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.

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