Scleroderma, also known as systemic sclerosis (SSc), is an autoimmune rheumato-logical disorder that causes inflammation, vascular damage, and fibrosis, and has a reported prevalence ranging from 7 per million to 489 per million persons. Geographic variations exist – SSc prevalence appears to be higher in the US (276 per million in 1990) and Australia, compared with Europe and Japan [1]. The incidence in the US is about 20 new cases per million adults per year [2]. SSc is a multisystem disease affecting a wide range of organs including the skin, lungs, kidneys, heart, and gastrointestinal (GI) tract. SSc is broadly classified as limited or diffuse cutaneous disease. In patients with limited cutaneous scleroderma (limited SSc), skin sclerosis is distal to the elbows and knees, with or without facial involvement. Patients may also have manifestations of the CREST syndrome (calcinosis cutis, Raynaud’s, esophageal dysmotility, sclerodactyly, and telangectasias), although CREST is not synonymous with limited SSc. Diffuse cutaneous scleroderma (diffuse SSc), in contrast, is characterized by thickening of the skin over the chest, abdomen, and proximal extremities in addition to involving distal areas. Patients with diffuse SSc are more likely to develop internal organ damage owing to ischemia or fibrosis, most often within the first 3 years of disease onset [3]. Nevertheless, it is important to consider that almost all cases will at some point have had less extensive skin involvement. Predictors of diffuse SSc in early disease include the presence of characteristic antibodies (anti Scl-70 and anti-RNA polymerase III antibodies) as well as tendon friction rubs. Tendon friction rubs, a “leathery crepitus” on palpation of the knees, wrists, fingers, and ankles during motion, are a significant predictor of the development of diffuse SSc [4,5]. Table 1 illustrates other key differences between limited and diffuse SSc.