Case Report Intractable colitis associated with chronic granulomatous disease

The case of a 20-year-old Japanese man, diagnosed as having autosomal recessive chronicgranulomatous disease (CGD), who was being treated with corticosteroids for intractableunclassified colitis, is described. He died from multiple organ failure following disseminatedintravascular coagulation secondary to disseminated varicella-zoster virus (VZV) infection. He wasdiagnosed as an index case of CGD when 2 years old, was inoculated against VZV at the ageof5 yearsandhadhadanunremarkablecoursefor19 years.Hewasadmittedtohospitalbecauseof a third episode of recurrent bloody diarrhoea. Clinical remission for each episode was achievedby intravenous corticosteroid therapy. Unclassified colitis associated with CGD was diagnosedbased on a colonic biopsy demonstrating characteristic macrophages with lipofuscin deposits.From a treatment viewpoint, idiopathic inflammatory bowel disease (IBD) should be differentiatedfrom secondary IBD occurring in CGD, in which immunosuppressive drugs includingcorticosteroids, still the mainstay of IBD treatment, should be avoided.