Diffuse cutaneous systemic sclerosis (dcSSc) is associated with high mortality resulting from early internal-organ involvement. Clinicians therefore tend to focus on early diagnosis and treatment of potentially life-threatening cardiorespiratory and renal disease. However, the rapidly progressive painful, itchy skin tightening that characterizes dcSSc is the symptom that has the greatest effect on patients’ quality of life, and there is currently no effective disease-modifying treatment for it. Considerable advances have been made in predicting the extent and rate of skin-disease progression (which vary between patients), including the development of techniques such as molecular analysis of skin biopsy samples. Risk stratification for progressive skin disease is especially relevant now that haematopoietic stem-cell transplantation is a treatment option, because stratification will inform the balance of risk versus benefit for each patient. Measurement of skin disease is a major challenge. Results from clinical trials have highlighted limitations of the modified Rodnan skin score (the current gold standard). Alternative patient-reported and other potential outcome measures have been and are being developed. Patients with early dcSSc should be referred to specialist centres to ensure best-practice management, including the management of their skin disease, and to maximize opportunities for inclusion in clinical trials.

弥漫性皮肤系统性硬化症 (dcSSc) 与早期内脏器官受累导致的高死亡率相关。因此，临床医生倾向于关注可能危及生命的心肺和肾脏疾病的早期诊断和治疗。然而，以 dcSSc 为特征的快速进展性疼痛、皮肤紧缩是对患者生活质量影响最大的症状，目前尚无有效的疾病缓解治疗方法。在预测皮肤病进展的程度和速度（因患者而异）方面已经取得了相当大的进步，包括皮肤活检样本分子分析等技术的发展。鉴于造血干细胞移植是一种治疗选择，进行性皮肤病的风险分层尤为重要，因为分层将告知每位患者风险与收益的平衡。皮肤病的测量是一项重大挑战。临床试验的结果突出了改良 Rodnan 皮肤评分（当前的黄金标准）的局限性。替代的患者报告的和其他潜在的结果测量已经并且正在开发中。早期 dcSSc 患者应转诊至专科中心，以确保最佳实践管理，包括皮肤病的管理，并最大限度地增加参与临床试验的机会。皮肤病的测量是一项重大挑战。临床试验的结果突出了改良 Rodnan 皮肤评分（当前的黄金标准）的局限性。替代的患者报告的和其他潜在的结果测量已经并且正在开发中。早期 dcSSc 患者应转诊至专科中心，以确保最佳实践管理，包括皮肤病的管理，并最大限度地增加参与临床试验的机会。皮肤病的测量是一项重大挑战。临床试验的结果突出了改良 Rodnan 皮肤评分（当前的黄金标准）的局限性。替代的患者报告的和其他潜在的结果测量已经并且正在开发中。早期 dcSSc 患者应转诊至专科中心，以确保最佳实践管理，包括皮肤病的管理，并最大限度地增加参与临床试验的机会。