Advances in diagnosis and treatment of juvenile localized scleroderma
10.3760/cma.j.issn.1673-4408.2022.10.014
- VernacularTitle:儿童局限性硬皮病的诊疗进展
- Author:
Caihui ZHANG
1
;
Hongmei SONG
Author Information
1. 中国医学科学院北京协和医学院北京协和医院儿科 100730
- Keywords:
Children;
Localized Scleroderma;
Diagnosis;
Treatment;
Pathogenesis
- From:
International Journal of Pediatrics
2022;49(10):708-712
- CountryChina
- Language:Chinese
-
Abstract:
Juvenile localized scleroderma is a rare childhood disease, the specific pathogenesis of this disease has not been fully clarified.Early recognition and timely diagnosis are crucial, early intervention is associated with good prognosis.But there is a lack of relatively reliable evaluation of disease activity and treatment response.At present, the main diagnostic basis of the disease is clinical manifestations.When there is doubt about the diagnosis, skin or subcutaneous tissue biopsy is required.In addition, high frequency Doppler ultrasound, shear-wave elastography, infrared thermal imaging, MRI, cone beam computed tomography, and dermatology evaluation tools, etc., all can reflect the patient′s condition to a certain extent.However, systematic evaluation should be improved in the process of diagnosis and treatment.In terms of treatment, hormones and methotrexate are still the first-line treatment for the disease, while evidence for second-line therapy is limited.Mycophenolate, hydroxychloroquine, cyclosporine A, tocilizumab and infliximab may play a certain role in the treatment of this disease.
