Abstract
Inflammatory linear verrucous epidermal naevus (ILVEN) is a rare congenital disease characterised by Blaschko-linear erythematous scaly skin. It is highly pruritic, disfiguring, and notoriously treatment-resistant. The genetic basis is heterogeneous: causes so far established are a single case of GJA1- and two of CARD14- mosaicism. However, clinical overlap with the skin lesions of CHILD syndrome led us to hypothesise that there may be a common biological pathway abnormality in ILVEN skin centred on cholesterol metabolic pathways.
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