Scleroderma is unique among our rheumatic diseases both in its clinical expression and its resistance to recovery when using our usual anti-inflammatory medications. The reason for this difference is now becoming apparent as the biology of the scleroderma process is better understood. It is now recognized that the disease expression is quite heterogeneous and that several different clinical but distinct phenotypes exists. This issue of "Rheumatic Disease Clinics" provides a concise update of developments in this area of study and covers topics such as vascular disease in scleroderma, diagnosis, management of the disease, genetics, novel treatments, scleroderma-like disorders, and its effect on the heart and lungs.