Scleroderma is a complex disease characterized by extensive fibrosis, vascular alterations, and autoantibodies against cellular antigens. There are two major subtypes: limited cutaneous scleroderma mainly affects the skin of the hands, arms, and face while diffuse cutaneous scleroderma rapidly affects a large area of skin and internal organs. The disease can lead to organ dysfunction and failure through heterogeneous mechanisms that are still unclear despite progress in treatment. Genetic factors contribute to susceptibility and different subtypes may have distinct autoantibody profiles associated with clinical features.