Summary

Common variable immunodeficiency (CVID) can show variant histological patterns in the gastrointestinal system. We present a case of an 11-year-old boy who has been followed up with a diagnosis of CVID since he was 6 months old. He presented with abdominal pain and diarrhoea. Colonoscopic biopsy showed crypt destructive colitis, severe decrease and focal absence of plasma cells. Three months later he suffered from abdominal pain, vomiting and bloody diarrhoea. Macroscopic examination of small intestinal resection material revealed multiple perforation areas, ulcers. Histopathology showed mild–moderate active enteritis with aphthous ulcers, purulent peritonitis, decrease in plasma cells and loss of primary follicles in lymph nodes. Histopathological findings were consistent with inflammatory bowel disease (IBD)-like CVID. Although in 6–10% of patients with CVID an IBD-like presentation is observed, this highly aggressive form is rarely seen. We present this case because of its extraordinary presentation displaying perforating active enteropathy.