Objective: To investigate the therapeutic effects of vitamine B 6 (Vit B 6) and Xuefu Zhuyu Decoction (血府逐瘀汤,XFZY, for activating blood circulation to remove stasis) in patients with localized scleroderma(LSD)....Objective: To investigate the therapeutic effects of vitamine B 6 (Vit B 6) and Xuefu Zhuyu Decoction (血府逐瘀汤,XFZY, for activating blood circulation to remove stasis) in patients with localized scleroderma(LSD). Methods: Thirty-three patients were treated with XFZY and Vit B 6, with 15 cases taking orally prednisone acetate and 20 healthy volunteers as the control. Their level of soluble interleukin-2 receptor (sIL-2R) and tumor necrosis factor-α (TNF-α) in the patients with LSD before and after treatment were observed. Results: The level of sIL-2R and TNF-α in the serum from the patients with LSD were higher than those of healthy volunteers ( P <0.01). After treatment with Vit B 6 and XFZY, the level of sIL-2R and TNF-α from the patients with LSD decreased significantly ( P <0.01), but there were no difference between the group taking Vit B 6 plus XFZY and the group given prednisone. Conclusion: The activating blood circulation to remove stasis approach in treating LSD with integrative Chinese and Western drugs got better results, and metabolic disorder of tryptophan might be correlated with the etiology of LSD.展开更多
Background:Juvenile Localized Scleroderma(JLS)is a rare pediatric rheumatic disease characterized by inflammation and skin sclerosis.The side effect of consensus-recommended medications and the risk of disability pose...Background:Juvenile Localized Scleroderma(JLS)is a rare pediatric rheumatic disease characterized by inflammation and skin sclerosis.The side effect of consensus-recommended medications and the risk of disability posed challenges to the JLS treatment.We intend to demonstrate the potential of traditional Chinese medicine in treating JLS with skin ulcers and reducing the dose of glucocorticoid.Method:Here we report a case of a 13-year-old male with JLS who took oral methotrexate tablets of 10 mg/week and methylprednisolone of 6 mg/day for over six months without significant effect and suffered from skin ulcers on the dorsal feet one month after drug cessation.Subsequently,the patient was treated with integrated traditional Chinese and Western medicine of low-dose glucocorticosteroid,adjusted Shenqi Huoxue formula and Jinshe Xiaoyan formula,etc.Results:After integrated treatment,the patient’s dorsal feet ulcers healed and the skin sclerosis and hyperpigmentation improved significantly.Conclusions:This case report suggests that integrated traditional Chinese and Western medicine can be used as an effective treatment for JLS.展开更多
Background Juvenile localized scleroderma(JLS)is a rare pediatric disease characterized by inflammation and skin thick ening.JLS is associated with deep tissue and extracutaneous involvement that often results in func...Background Juvenile localized scleroderma(JLS)is a rare pediatric disease characterized by inflammation and skin thick ening.JLS is associated with deep tissue and extracutaneous involvement that often results in functional impairment and growth disturbances.This article provides an overview of the disease with a focus on active features and treatment.Data sources We searched databases including PubMed,Elsevier and MedLine and Wanfang,reviewing publications from 2013 to 2019.Selected earlier publications were also reviewed.Results Linear scleroderma is the most common JLS subtype.Several lines of evidence suggest that JLS is an autoimmune disease.Extracutaneous involvement is common and can present before the onset of skin disease.Multiple skin features are associated with disease activity,and activity can also manifest as arthritis,myositis,uveitis,seizures,and growth impairment.Systemic immunosuppressive treatment,commonly methotrexate with or without glucocorticoids,greatly improves outcome and is recommended for treating JLS patients with active disease and moderate or higher severity.Long term monitoring is needed because of the disease's chronicity and the high frequency of relapses off of treatment.Conclusions JLS is associated with a risk for disabling and disfiguring morbidity for the growing child.Identifying active disease is important for guiding treatment,but often difficult because of the paucity of markers and lack of a universal skin activity feature.More studies of JLS pathophysiology are needed to allow the identification of biomarkers and therapeutic targets.Comparative effectiveness treatment studies are also needed to work towards optimizing care and outcome.展开更多
Localized Scleroderma, also known as morphea, is a dermal disease manifested by localized edematous skin primarily and progressive thickening and hardening of the skin. It mainly affects the scalp, forehead, lumbar, a...Localized Scleroderma, also known as morphea, is a dermal disease manifested by localized edematous skin primarily and progressive thickening and hardening of the skin. It mainly affects the scalp, forehead, lumbar, abdomen and four limbs. The skin lesions are firstly present in single or multiple pink edematous patches in varying sizes, and then change into primrose yellow or beige hardening, being smooth and shiny resembling wax.展开更多
Pathological scarring and scleroderma,which are the most common conditions of skin fibrosis,pathologically manifest as fibroblast proliferation and extracellular matrix(ECM)hyperplasia.Fibroblast proliferation and ECM...Pathological scarring and scleroderma,which are the most common conditions of skin fibrosis,pathologically manifest as fibroblast proliferation and extracellular matrix(ECM)hyperplasia.Fibroblast proliferation and ECM hyperplasia lead to fibrotic tissue remodeling,causing an exaggerated and prolonged wound-healing response.The pathogenesis of these diseases has not been fully clarified and is unfortunately accompanied by exceptionally high medical needs and poor treatment effects.Currently,a promising and relatively low-cost treatment has emerged-adipose-derived stem cell(ASC)therapy as a branch of stem cell therapy,including ASCs and their derivatives-purified ASC,stromal vascular fraction,ASC-conditioned medium,ASC exosomes,etc.,which are rich in sources and easy to obtain.ASCs have been widely used in therapeutic settings for patients,primarily for the defection of soft tissues,such as breast enhancement and facial contouring.In the field of skin regeneration,ASC therapy has become a hot research topic because it is beneficial for reversing skin fibrosis.The ability of ASCs to control profibrotic factors as well as anti-inflammatory and immunomodulatory actions will be discussed in this review,as well as their new applications in the treatment of skin fibrosis.Although the long-term effect of ASC therapy is still unclear,ASCs have emerged as one of the most promising systemic antifibrotic therapies under development.展开更多
Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapi...Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapies are limited and insufficiently treat the cutaneous manifestations of scleroderma.Autologous fat transfer(AFT)is a surgical technique that has been utilized for many decades for facial rejuvenation.The adipose stem cells(ASCs)present in fat grafts have also shown significant promise for their anti-inflammatory and regenerative properties.Recently,AFT has been repurposed to treat the skin manifestations of systemic sclerosis and localized scleroderma.Studies suggest that AFT in scleroderma patients improves mouth and hand functions,Raynaud’s symptoms,and digital ulcerations.AFT is a safe procedure with rare postoperative complications,making it a promising intervention for the treatment of scleroderma.Further studies are required to better characterize the influence of fat grafts on the recipient site and to establish standards for fat transfer in fibrotic skin diseases.展开更多
文摘Objective: To investigate the therapeutic effects of vitamine B 6 (Vit B 6) and Xuefu Zhuyu Decoction (血府逐瘀汤,XFZY, for activating blood circulation to remove stasis) in patients with localized scleroderma(LSD). Methods: Thirty-three patients were treated with XFZY and Vit B 6, with 15 cases taking orally prednisone acetate and 20 healthy volunteers as the control. Their level of soluble interleukin-2 receptor (sIL-2R) and tumor necrosis factor-α (TNF-α) in the patients with LSD before and after treatment were observed. Results: The level of sIL-2R and TNF-α in the serum from the patients with LSD were higher than those of healthy volunteers ( P <0.01). After treatment with Vit B 6 and XFZY, the level of sIL-2R and TNF-α from the patients with LSD decreased significantly ( P <0.01), but there were no difference between the group taking Vit B 6 plus XFZY and the group given prednisone. Conclusion: The activating blood circulation to remove stasis approach in treating LSD with integrative Chinese and Western drugs got better results, and metabolic disorder of tryptophan might be correlated with the etiology of LSD.
基金The essay is supported by Research Project of Shanghai Municipal Health Care Commission,No.20204Y0410We appreciate the patient and his parents for their cooperation and consent to disclose the case.We express our gratitude for the support from the members of the scientific innovation volunteer team of rare diseases in Shanghai TCM-Integrated School of clinical medicine,Shanghai University of Traditional Chinese Medicine.
文摘Background:Juvenile Localized Scleroderma(JLS)is a rare pediatric rheumatic disease characterized by inflammation and skin sclerosis.The side effect of consensus-recommended medications and the risk of disability posed challenges to the JLS treatment.We intend to demonstrate the potential of traditional Chinese medicine in treating JLS with skin ulcers and reducing the dose of glucocorticoid.Method:Here we report a case of a 13-year-old male with JLS who took oral methotrexate tablets of 10 mg/week and methylprednisolone of 6 mg/day for over six months without significant effect and suffered from skin ulcers on the dorsal feet one month after drug cessation.Subsequently,the patient was treated with integrated traditional Chinese and Western medicine of low-dose glucocorticosteroid,adjusted Shenqi Huoxue formula and Jinshe Xiaoyan formula,etc.Results:After integrated treatment,the patient’s dorsal feet ulcers healed and the skin sclerosis and hyperpigmentation improved significantly.Conclusions:This case report suggests that integrated traditional Chinese and Western medicine can be used as an effective treatment for JLS.
文摘Background Juvenile localized scleroderma(JLS)is a rare pediatric disease characterized by inflammation and skin thick ening.JLS is associated with deep tissue and extracutaneous involvement that often results in functional impairment and growth disturbances.This article provides an overview of the disease with a focus on active features and treatment.Data sources We searched databases including PubMed,Elsevier and MedLine and Wanfang,reviewing publications from 2013 to 2019.Selected earlier publications were also reviewed.Results Linear scleroderma is the most common JLS subtype.Several lines of evidence suggest that JLS is an autoimmune disease.Extracutaneous involvement is common and can present before the onset of skin disease.Multiple skin features are associated with disease activity,and activity can also manifest as arthritis,myositis,uveitis,seizures,and growth impairment.Systemic immunosuppressive treatment,commonly methotrexate with or without glucocorticoids,greatly improves outcome and is recommended for treating JLS patients with active disease and moderate or higher severity.Long term monitoring is needed because of the disease's chronicity and the high frequency of relapses off of treatment.Conclusions JLS is associated with a risk for disabling and disfiguring morbidity for the growing child.Identifying active disease is important for guiding treatment,but often difficult because of the paucity of markers and lack of a universal skin activity feature.More studies of JLS pathophysiology are needed to allow the identification of biomarkers and therapeutic targets.Comparative effectiveness treatment studies are also needed to work towards optimizing care and outcome.
文摘Localized Scleroderma, also known as morphea, is a dermal disease manifested by localized edematous skin primarily and progressive thickening and hardening of the skin. It mainly affects the scalp, forehead, lumbar, abdomen and four limbs. The skin lesions are firstly present in single or multiple pink edematous patches in varying sizes, and then change into primrose yellow or beige hardening, being smooth and shiny resembling wax.
基金Supported by National Natural Science Foundation of China,No.81772103 and No.82172234Shanghai Clinical Research Center of Plastic and Reconstructive Surgery supported by Science and Technology Commission of Shanghai Municipality,China,No.22MC1940300。
文摘Pathological scarring and scleroderma,which are the most common conditions of skin fibrosis,pathologically manifest as fibroblast proliferation and extracellular matrix(ECM)hyperplasia.Fibroblast proliferation and ECM hyperplasia lead to fibrotic tissue remodeling,causing an exaggerated and prolonged wound-healing response.The pathogenesis of these diseases has not been fully clarified and is unfortunately accompanied by exceptionally high medical needs and poor treatment effects.Currently,a promising and relatively low-cost treatment has emerged-adipose-derived stem cell(ASC)therapy as a branch of stem cell therapy,including ASCs and their derivatives-purified ASC,stromal vascular fraction,ASC-conditioned medium,ASC exosomes,etc.,which are rich in sources and easy to obtain.ASCs have been widely used in therapeutic settings for patients,primarily for the defection of soft tissues,such as breast enhancement and facial contouring.In the field of skin regeneration,ASC therapy has become a hot research topic because it is beneficial for reversing skin fibrosis.The ability of ASCs to control profibrotic factors as well as anti-inflammatory and immunomodulatory actions will be discussed in this review,as well as their new applications in the treatment of skin fibrosis.Although the long-term effect of ASC therapy is still unclear,ASCs have emerged as one of the most promising systemic antifibrotic therapies under development.
文摘Scleroderma is a chronic connective tissue disease characterized by inflammation,vascular injury,and progressive skin fibrosis,resulting in significant aesthetic and functional impairments for patients.Current therapies are limited and insufficiently treat the cutaneous manifestations of scleroderma.Autologous fat transfer(AFT)is a surgical technique that has been utilized for many decades for facial rejuvenation.The adipose stem cells(ASCs)present in fat grafts have also shown significant promise for their anti-inflammatory and regenerative properties.Recently,AFT has been repurposed to treat the skin manifestations of systemic sclerosis and localized scleroderma.Studies suggest that AFT in scleroderma patients improves mouth and hand functions,Raynaud’s symptoms,and digital ulcerations.AFT is a safe procedure with rare postoperative complications,making it a promising intervention for the treatment of scleroderma.Further studies are required to better characterize the influence of fat grafts on the recipient site and to establish standards for fat transfer in fibrotic skin diseases.