Congenital hemidysplasia with ichthyosiform naevus and limb defects (CHILD) syndrome is an X-linked dominant disorder with fatality for male embryos (1, 2). The disorder is caused by mutations in NSDHL (NAD[P]

© 2015 The Authors. doi: 10.2340/00015555-1859 Journal Compilation © 2015 Acta Dermato-Venereologica. ISSN 0001-5555 Congenital hemidysplasia with ichthyosiform naevus and limb defects (CHILD) syndrome is an X-linked dominant disorder with fatality for male embryos (1, 2). The disorder is caused by mutations in NSDHL (NAD[P] H steroid dehydrogenase-like protein), which is of importance in the cholesterol biosynthetic pathway (3). CHILD naevus, representing a peculiar inflammatory keratinocytic naevus, is a characteristic feature of CHILD syndrome (1). In most cases, this skin disorder shows a distinctive lateralisation pattern with a strict midline demarcation and affinity to body folds (4, 5). Recently, new therapeutic approaches based on the pathogenesis have been developed (6, 7). A body of evidences show that ketoconazole can influence cholesterol biosynthesis via cytochrome P450 enzyme (8); that ketoconazole might thus be a promising agent to the cutaneous lesions of congenital ichthyoses induced by cholesterol metabolic disturbances.