Lack of effect of topical statins in linear porokeratosis.

Linear porokeratosis (LP) is a mosaic disorder of keratinisation caused by second-hit postzygotic mutations in the presence of monoallelic germline mutations in genes encoding enzymes of the mevalonate pathway, MVD, MVK or PMVK. Widespread LP can cause a high disease burden and represents a therapeutic challenge. The pathogenesis of LP implies anomalies of cholesterol biosynthesis, and therefore a pathogenesis-based therapy with statins to prevent accumulation of precursors in the skin, seems meaningful. Recently, a lovastatin/cholesterol cream was shown to be effective in disseminated porokeratosis, however the outcome in two patients with LP was poor.