Rapid improvement of skin lesions in CHILD syndrome with topical 5% simvastatin ointment

Abstract

Congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD) syndrome is a rare X‐linked dominant disorder of cholesterol synthesis characterized by unilateral ichthyosiform dermatitis with ipsilateral limb hypoplasia. Recently, pathogenesis‐based treatment has demonstrated improvement of skin lesions with statins by decreasing formation of cholesterol intermediates through inhibition of cholesterol synthesis. We report a 10‐month‐old girl who presented with unilateral scaly ptychotropic plaques, who experienced rapid, near‐complete clearance with topical 5% simvastatin monotherapy twice daily.

New developments in the molecular treatment of ichthyosis: review of the literature

“…Skin lesions slightly improved after treatment with 2.5% simvastatin, but a marked improvement was seen after switching to 5% simvastatin. Other case reports of simvastatin monotherapy show similar responses [ 90 , 91 ].…”

“…[ 89 ] Case series CHILD syndrome Cholesterol replacement; 2.5% and 5% simvastatin 2022 Kallis et al . [ 90 ] Case report CHILD syndrome Cholesterol replacement; 5% simvastatin 2018 Bajawi et al . [ 91 ] Case report CHILD syndrome Cholesterol replacement; 2% simvastatin 2019 Di et al .…”

Congenital Ichthyosis: A Practical Clinical Guide on Current Treatments and Future Perspectives

“…13 Cases of congenital hemidysplasia with ichthyosiform erythroderma and limb defects (CHILD syndrome) have had the skin changes improved with the application of a compounded topical statin. 14,15 Individuals with NS that have associated primary immunodeficiency may experience cutaneous improvement with immunoglobulin replacement therapy. 16 After finding that adults with epidermolytic ichthyosis, NS, lamellar ichthyosis, or congenital ichthyosiform erythroderma with at least moderate erythroderma had IL-17A immune skewing, a cohort of 20 subjects were treated with secukinumab with heterogeneous results.…”