To review the differences in the epidemiology, clinical manifestations, and severity of systemic sclerosis in different ethnic groups. This review considers only limited and diffuse forms of systemic sclerosis. The most relevant articles about ethnic differences in systemic sclerosis were selected. The information is presented in three sections: differences in the epidemiology of systemic sclerosis between ethnic groups; differences in clinical manifestations between ethnic group, and factors that may explain these differences. Of the different ethnic groups studied, whites have less incidence and prevalence of systemic sclerosis. Whites also have less frequency of inflammatory manifestations, and have a higher frequency of limited skin disease compared with other ethnic groups. These differences may result in the better survival of whites compared with no whites. The origin of these differences has not been discovered so far. Is probable that genetic and environmental factors may play a role in the expression of disease, producing the differences found among the ethnic groups.