Application of integrated traditional Chinese and Western medicine in the treatment of juvenile localized scleroderma with skin ulcer: a case report

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.

Overview of juvenile localized scleroderma and its management

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.