Porokeratosis encompasses a group of keratinization disorders with distinct clinical variants,including porokeratosis of Mibelli,disseminated superficial actinic porokeratosis,porokeratosis plantaris,palmaris et disse...Porokeratosis encompasses a group of keratinization disorders with distinct clinical variants,including porokeratosis of Mibelli,disseminated superficial actinic porokeratosis,porokeratosis plantaris,palmaris et disseminata,and linear porokeratosis(LP).1 Familial porokeratosis has been associated with pathogenic variants in genes of the mevalonate pathway(such as MVK,PMVK,MVD,and FDPS),a vital metabolic pathway responsible for synthesizing sterols and isoprenoid metabolites1-3.展开更多
Atopic dermatitis(AD)is a complex disease characterized by recurrent eczematous lesions and refractory pruritus that drastically impairs quality of life.Due to the chronic and relapsing course,patients are easily trap...Atopic dermatitis(AD)is a complex disease characterized by recurrent eczematous lesions and refractory pruritus that drastically impairs quality of life.Due to the chronic and relapsing course,patients are easily trapped in the debilitating condition.Classical therapies show limitations,especially for patients with moderate-to-severe phenotypes.Advanced new insights in targeted therapies exhibit great application prospects which were reinforced by the more profound understanding of the disease pathogenesis.However,the sustained efficiency,biosafety,and long-term benefits still remain in further exploration.This review summarizes recent clinical studies on oral small-molecule inhibitors and biological agents for pediatric AD patients,which provides the latest frontiers to clinicians.展开更多
To the editor:Incontinentia pigmenti(IP)is a rare X-linked dominant neuroectodermal disorder primarily caused by mutations in the IKBKG(NEMO)gene.Cutaneous manifestations of IP typically progress through four stages:i...To the editor:Incontinentia pigmenti(IP)is a rare X-linked dominant neuroectodermal disorder primarily caused by mutations in the IKBKG(NEMO)gene.Cutaneous manifestations of IP typically progress through four stages:inflammatory(or vesicular)stage,verrucous stage,hyperpigmented stage,and hypopigmented(or atropic)stage.^(1)Throughout the disease course,most cases develop linear or whorled brownish reticular pigmentation following the lines of Blaschko.Additionally,some patients experience linear hypopigmentation and absence of hair,usually during adolescence.Here,we report a rare manifestation of this disease.展开更多
基金supported by grants from the National Natural Science Foundation of China(No.82073422,82273504).
文摘Porokeratosis encompasses a group of keratinization disorders with distinct clinical variants,including porokeratosis of Mibelli,disseminated superficial actinic porokeratosis,porokeratosis plantaris,palmaris et disseminata,and linear porokeratosis(LP).1 Familial porokeratosis has been associated with pathogenic variants in genes of the mevalonate pathway(such as MVK,PMVK,MVD,and FDPS),a vital metabolic pathway responsible for synthesizing sterols and isoprenoid metabolites1-3.
文摘Atopic dermatitis(AD)is a complex disease characterized by recurrent eczematous lesions and refractory pruritus that drastically impairs quality of life.Due to the chronic and relapsing course,patients are easily trapped in the debilitating condition.Classical therapies show limitations,especially for patients with moderate-to-severe phenotypes.Advanced new insights in targeted therapies exhibit great application prospects which were reinforced by the more profound understanding of the disease pathogenesis.However,the sustained efficiency,biosafety,and long-term benefits still remain in further exploration.This review summarizes recent clinical studies on oral small-molecule inhibitors and biological agents for pediatric AD patients,which provides the latest frontiers to clinicians.
基金supported by the National Nature Science Foundation(82073422 and 82273504)the Shanghai Municipal Natural Science Foundation(22ZR1440800).
文摘To the editor:Incontinentia pigmenti(IP)is a rare X-linked dominant neuroectodermal disorder primarily caused by mutations in the IKBKG(NEMO)gene.Cutaneous manifestations of IP typically progress through four stages:inflammatory(or vesicular)stage,verrucous stage,hyperpigmented stage,and hypopigmented(or atropic)stage.^(1)Throughout the disease course,most cases develop linear or whorled brownish reticular pigmentation following the lines of Blaschko.Additionally,some patients experience linear hypopigmentation and absence of hair,usually during adolescence.Here,we report a rare manifestation of this disease.
