5-lipoxygenase inhibition: A new treatment strategy for Sjogren-Larsson syndrome

The Sjogren-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 B-4 (LTB4) opened the way to the development of a new therapeutic strategy for SLS, i.e. 5-lipoxy genase inhibition. We treated one SLS patient with zileuton during five wee ks. 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 pea k" of cerebral white matter (that is characteristically found on proton mag netic resonance spectroscopy in SLS patients) decreased during treatment, a nd increased again when treatment was stopped. In conclusion, the beneficia l effects of 5-lipoxygenase inhibition in SLS are very promising and encour age further research.