Background: ABO blood group antigens may play a role in the pathophysiology of certain diseases. Several studies have investigated the relation between blood group and autoimmune disorders. In this research, we evaluated the associations between ABO blood types and rhesus (Rh) factor in pemphigus patients.Methods: In this case-control, hospital-based study, we evaluated the prevalence of blood group on 110 pemphigus patients and 126 controls. The chi-square test was used to compare qualitative variables and examine the relationship between blood groups and Rh in pemphigus patients and a control group.Results: Blood group (O) was found in 46.3% (O+ 38.2%) of patients with pemphigus, followed by blood type A (30.0%; A+ 27.3%), and blood groups B and AB. Compared to the control group, we noted that the distribution of blood groups, with Rh (P=0.73) or without Rh (P=0.52), did not significantly differ.Conclusion: Although some associations between blood group antigens and specific diseases have been reported in the literature, this study did not find any statistically significant relationship between blood groups and pemphigus disease.