Related Experiment Video
Updated: Oct 16, 2026

A Lip Fixation Device Facilitates Labial Salivary Gland Biopsy for Sjögren's Disease
Published on: March 6, 2026
5-Lipoxygenase inhibition: a new treatment strategy for Sjögren-Larsson syndrome
M A Willemsen1, J J Rotteveel, P M Steijlen
1Department of Pediatric Neurology, University Hospital Nijmegen, The Netherlands.
Abstract:
The Sjögren-Larsson syndrome (SLS) is a severe neurocutaneous disorder due to fatty aldehyde dehydrogenase (FALDH) deficiency. The recent discovery of the role of FALDH in the degradation of leukotriene B4 (LTB4) opened the way to the development of a new therapeutic strategy for SLS, i.e. 5-lipoxygenase inhibition. We treated one SLS patient with zileuton during five weeks. During the treatment period we found decreased values of LTB4 and omega-OH-LTB4. The severity of the pruritus diminished, and favorable changes in the child's behavior were observed. The height of the prominent "lipid peak" of cerebral white matter (that is characteristically found on proton magnetic resonance spectroscopy in SLS patients) decreased during treatment, and increased again when treatment was stopped. In conclusion, the beneficial effects of 5-lipoxygenase inhibition in SLS are very promising and encourage further research.
Related Concept Videos
Antiasthma Drugs: Leukotriene Modifiers
Leukotriene modifiers work through two distinct mechanisms:
Inflammatory Bowel Disease IV: Pharmacological Management
Pharmacologic...
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Lipid-Lowering Drugs: Statins and Miscellaneous Agents
Drugs for Treatment of Crohn's Disease in IBD Using Immunomodulatory Agents
Open Angle Glaucoma: Treatment
Drugs such as carbonic anhydrase inhibitors, α2- and...