Systemic sclerosis is a generalized disorder characterized by fibrosis and microvascular injury in affected organs. Despite being recognized nearly 250 years ago, knowledge regarding pathogenesis remains limited, and treatment remains directed at symptomatic improvement. Early recognition of systemic sclerosis, however, is important in order to monitor for specific disease complications (i.e., fibrosing alveolitis, scleroderma renal crisis) as well as initiate manifestation specific therapies that improve quality of life.