Clinical features and evaluation of systemic sclerosis: A dermatology-based case series
Keywords:
Connective tissue disease, Raynaud’s phenomenon, SclerodermaAbstract
Background: Systemic sclerosis (SSc) is a rare, complex immune mediated connective tissue disease characterized by progressive skin fibrosis and other clinically heterogenous features. Timely referral and early diagnosis of organ manifestations are essential to allow intervention before organ damage occurs. Case summary: Three patients (2 females and 1 male), aged 23, 34, and 48 years old were identified with a diagnosis of SSc. Duration of the disease was 3 to 5 years with absence of SSc positive family history. Each patient had a clinically SSc with characteristic cutaneous findings according to the ACR/EULAR (American College of Rheumatology/European League against Rheumatism) 2013 diagnostic criteria. All patients had skin thickening of the fingers, abnormal nail-fold capillaroscopy, and Raynaud’s phenomenon. One patient had a non-trauma related digital tip ulcer and another patient presented with facial telangiectasia. SSc related autoantibody (anti-topoisomerase I) was detected in 2 patients while the remaining 1 patient was positive for anti RNP/Sm. Non-SSc related Anti-Ro/ SSA autoantibodies were also detected in 2 patients. Histopathology examination of each patient were consistent with SSc. Patients were multi-specialties managed due to the multifaceted clinical manifestations of SSc. Conclusion: A wide spectrum of clinical phenotypes and autoantibodies of SSc may be observed. The skin provides a window to systemic progression in SSc, allowing dermatologists to contribute to early diagnosis, treatment initiation, effective disease monitoring, and improved patient outcomes.References
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