An excellent response to topical therapy of four congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome patients with an increased concentration of simvastatin ointment

Xia, Yu; Chen, L; Yang, Zhong-Jin; Gu, Yaoyao; Zheng, Weiwei; Wu, Zhouwei; Li, M.; Yao, Zhirong

doi:10.1111/jdv.15838

Cited by 4 publications

(8 citation statements)

References 8 publications

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“…HMG CoA reductase inhibitors target the rate‐limiting enzyme of cholesterol biosynthesis and have been utilized topically in combination with cholesterol to mitigate formation of toxic metabolites in CHILD syndrome 1‐3 . Repletion of cholesterol alone is insufficient for the treatment of cutaneous lesions 2 ; accumulation of toxic sterol metabolites may have a greater negative impact on keratinization than cholesterol deficiency 4,5 . Our experience using compounded topical 5% simvastatin without cholesterol corroborates recent reports 4,5 that topical statin monotherapy can successfully treat skin lesions in CHILD syndrome.…”

Section: Discussionsupporting

confidence: 77%

“…Recent therapeutic developments focus on replacing the underlying cholesterol deficiency and decreasing accumulation of cholesterol precursors 4 . Successful treatments have included a combination of topical statins and cholesterol in varying concentrations, as well as statin monotherapy 1‐5 . We report a case of a 10‐month‐old otherwise healthy girl with progressive ptychotropic ichthyosiform erythroderma who demonstrated rapid improvement with topical simvastatin 5% ointment.…”

Section: Introductionmentioning

confidence: 99%

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

Kallis

Bisbee

Garganta

et al. 2021

Pediatric Dermatology

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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.

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Section: Discussionsupporting

confidence: 77%

Section: Introductionmentioning

confidence: 99%

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

Kallis

Bisbee

Garganta

et al. 2021

Pediatric Dermatology

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“…1a). We increased the concentration of simvastatin to 5%, as it was reported to be more effective 5 . This brought some benefit up to 10 months of follow‐up, and the patient experienced less itch and pain than before.…”

Section: Figurementioning

confidence: 99%

Lack of effect of topical statins in linear porokeratosis

Saleva-Stateva

Weibel

Theiler

et al. 2020

Acad Dermatol Venereol

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“…Simvastatin is also an inhibitor for HMG‐CoA reductase. Our previous study provided some modifications to the treatment medicine, demonstrating that 2.5% and 5% simvastatin ointment were both effective and safe in the treatment of four cases with CHILD syndrome, and 5% simvastatin ointment achieved better improvement 5 …”

Section: Introductionmentioning

confidence: 99%

“…Our previous study provided some modifications to the treatment medicine, demonstrating that 2.5% and 5% simvastatin ointment were both effective and safe in the treatment of four cases with CHILD syndrome, and 5% simvastatin ointment achieved better improvement. 5 Here, we report a study of seven DPX patients treated with 1% or 5% simvastatin ointment based on the common pathology of CHILD syndrome, and further assess their efficacy and tolerance.…”

Section: Introductionmentioning

confidence: 99%

Simvastatin ointment in the treatment of seven childhood diffuse plane xanthomas

Xia

Yang

et al. 2020

The Journal of Dermatology

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Diffuse plane xanthomas (DPX) is a rare, chronic, metabolic skin disease which can seriously affect the appearance of the patients and result in psychological problems. Existing treatments are seriously limited. Our objective was to assess the efficacy of and tolerance to simvastatin ointment for the treatment of skin lesions in seven DPX patients. In this study, patients were treated twice daily on the right side of the lesions for 10 months with the 1% or 5% simvastatin ointment. Meanwhile, the left body was untreated as a contralateral controlled side. Efficacy was assessed via the change of rash and was further confirmed by histological study. For all seven participants, both the 1% and 5% simvastatin ointments were effective. The histological change in DPX showed significant decrease of foam cells in skin lesions. The immunohistochemical staining of CD68, macrophage scavenger receptor type 1, oxidized low‐density lipoprotein receptor 1 and Oil Red O became obviously reduced after treatment. The test medicines were safe with only some skin‐related side‐effects. This is the first research reported on simvastatin ointment treatment in DPX. Our results are the first to suggest that simvastatin ointment is effective in improving skin lesions of DPX clinically and histopathologically.

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An excellent response to topical therapy of four congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome patients with an increased concentration of simvastatin ointment

Cited by 4 publications

References 8 publications

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

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

Lack of effect of topical statins in linear porokeratosis

Simvastatin ointment in the treatment of seven childhood diffuse plane xanthomas

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