Cutaneous presentations of systemic sclerosis in skin of colour: A systematic review

Abstract

Systemic sclerosis (SSc), a rheumatologic autoimmune disease, can present diversely, especially in patients with skin of colour (SOC). Differences in clinical manifestations among SOC individuals compared to predominantly White populations require a comprehensive examination to optimize diagnosis and treatment. This systematic review aims to explore the differences in SSc manifestations among individuals with SOC, identifying variations in disease severity, clinical features and psychosocial impacts. A systematic review of the literature was conducted following PRISMA guidelines. Databases searched included PubMed, SCOPUS, Cochrane and Web of Science, focusing on studies published within the last 10 years. Nine studies were selected and qualitatively analysed due to the heterogeneity of study types. Key findings highlight significant variations in disease severity and cutaneous clinical features among SOC cohorts compared to predominantly White populations. Raynaud's phenomenon was prevalent across all ethnicities; however, SOC groups exhibited fewer hallmark features of scleroderma, such as telangiectasias, calcinosis and digital swelling, which complicates diagnosis. SOC individuals showed a higher propensity for severe pulmonary complications and increased mortality rates. Atypical cutaneous presentations in SOC were associated with disease progression. Psychosocial impacts were more pronounced in SOC individuals, with greater awareness and dissatisfaction over cutaneous symptoms. Recognizing the atypical presentations of SSc in SOC is critical for early diagnosis and optimizing patient outcomes. The study underscores the need for further research to determine if observed differences are due to variations in prevalence, genetic factors or insufficient training in recognizing cutaneous manifestations in SOC. Enhanced awareness and targeted training for healthcare providers are essential to address these diagnostic challenges and improve care for SOC patients with SSc.