Lupus Erythematosus and Chronic Granulomatous Disease: Report of Four Iranian Patients with AR-CGD and One XL-CGD

  • 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
Keywords: Chronic, Cutaneous, Granulomatous disease, Iran, Lupus erythematosus, Rare diseases

Abstract

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.

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Published
2019-08-17
How to Cite
1.
Maddah M, Fazlollahi MR, Shiari R, Shahram F, Mamishi S, Babaie D, Monajemzadeh M, Sotudeh S, Hamidieh AA, Badalzadeh M, Tajik S, Sedighipour L, Pourpak Z. Lupus Erythematosus and Chronic Granulomatous Disease: Report of Four Iranian Patients with AR-CGD and One XL-CGD. Iran J Allergy Asthma Immunol. 18(4):452-458.
Section
Case Series