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

Iran J Allergy Asthma Immunol. 2019 Aug 17;18(4):452-458. doi: 10.18502/ijaai.v18i4.1426.

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.

Keywords: Chronic; Cutaneous; Granulomatous disease; Iran; Lupus erythematosus; Rare diseases.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Alleles
  • Biomarkers
  • Biopsy
  • Child
  • Disease Susceptibility
  • Female
  • Genes, Recessive*
  • Genes, X-Linked*
  • Genotype
  • Granulomatous Disease, Chronic / diagnosis*
  • Granulomatous Disease, Chronic / etiology*
  • Humans
  • Iran
  • Lupus Erythematosus, Systemic / complications*
  • Lupus Erythematosus, Systemic / genetics*
  • Male
  • Phenotype
  • Young Adult

Substances

  • Biomarkers