Vedolizumab use for severe chronic enterocolitis in a child with Hoyeraal–Hreidarsson syndrome: A case report

We report a patient with Hoyeraal–Hreidarsson syndrome (HHS), a telomere biology disorder, who developed severe chronic enterocolitis with inflammatory bowel disease‐like features. Vedolizumab led to improvement in stool frequency, reduced fecal calprotectin, and clinical remission without infectious complications. Remission was maintained with vedolizumab monotherapy for 2 years after discontinuation of budesonide and parenteral nutrition. This report highlights vedolizumab as a potentially safe therapeutic option for enterocolitis in HHS, emphasizing the value of gut‐selective therapy when systemic immunosuppression is risky. Further studies are needed to establish the long‐term efficacy and safety of vedolizumab in patients with telomere biology disorders.