Tehran University of Medical Sciences Iranian Journal of Allergy, Asthma and Immunology 1735-1502 18 4 2019 08 17 452 458 EN Marzieh Maddah Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran Mohammad Reza Fazlollahi Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran Reza Shiari Department of Pediatric Rheumatology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Farhad Shahram Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran Setareh Mamishi Department of Infectious Diseases, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Delara Babaie Department of Allergy and Clinical Immunology, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Maryam Monajemzadeh Department of Pathology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Soheila Sotudeh Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Amir Ali Hamidieh Department of Pediatric Stem Cell Transplantation, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Mohsen Badalzadeh Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran Shaghayegh Tajik Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran Leila Sedighipour Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran Zahra Pourpak Immunology, Athma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran 2017 09 27 2018 11 04 Chronic granulomatous disease (CGD) is a rare genetic disorder of neutrophil activity, resulting in increased rate of recurrent infections with catalase–positive bacteria and fungi, as well as various autoimmune diseases such as sarcoidosis, rheumatoid arthritis, and discoid and/or systemic lupus erythematosus. Few reports have reported lupus erythematosus (LE) in patients with X–linked CGD (XL-CGD) and carriers, and very few in autosomal recessive CGD (AR-CGD). Here, we present 5 patients with CGD developing LE at different ages to emphasize on the importance of appropriate follow–up and treatment in patients with CGD with clinical signs and symptoms of autoimmune diseases and even in those with negative serologic results. https://ijaai.tums.ac.ir/index.php/ijaai/article/view/1671 https://ijaai.tums.ac.ir/index.php/ijaai/article/download/1671/1433