Abstract:
Localized scleroderma (LoS) is a disease characterized by inflammation, sclerosis, and atrophy of the skin and subcutaneous tissues. It may involve deeper structures such as subcutaneous fat, fascia, muscle, and bone, as well as extracutaneous structures including the eyes, nervous system, and craniofacial region. Some patients may develop atrophic deformities, leading to functional impairment and cosmetic concerns. This guideline was developed through systematic literature review, evidence synthesis, and integration with current clinical practice in China, followed by multiple rounds of multidisciplinary expert discussion, revision, and consensus achieved using a modified Delphi method. The guideline provides recommendations on epidemiology, etiology and pathogenesis, clinical classification, diagnosis and differential diagnosis, laboratory and imaging assessment, evaluation of disease activity and severity, treatment principles and therapeutic approaches, and long-term follow-up of LoS. As the first national guideline in China for the diagnosis and management of LoS, it emphasizes stratified assessment and individualized management based on disease activity, severity, depth and location of involvement, as well as the risk of functional and cosmetic impairment. Multidisciplinary management is recommended for high-risk patients, including those with deep tissue involvement, craniofacial linear lesions, or musculoskeletal and neurological involvement. This guideline aims to support standardized diagnosis, treatment, follow-up, and comprehensive management of LoS in clinical practice in China, and will be updated regularly in accordance with emerging evidence.