Dual Biologic Therapy in an Adolescent With Camurati-Engelmann Disease and Crohn Disease

This case report describes a 13-year-old girl with Camurati-Engelmann disease (CED) and Crohn disease (CD) who was referred for hematochezia and diarrhea. After infliximab improved gastrointestinal symptoms but worsened CED-associated bone pain, vedolizumab was initiated and provided initial improvement without bone pain exacerbation. Hematochezia recurred after the fourth vedolizumab dose, and the patient developed severe pyoderma gangrenosum. Ustekinumab was added as a second biologic. By 6 months of dual biologic therapy with vedolizumab and ustekinumab, clinical and biochemical remission were achieved with resolution of pyoderma gangrenosum and substantial weight gain. Body mass index increased by 41% over the first year of dual biologic therapy, and bone pain remained well controlled. This single case report cannot establish efficacy of dual biologic therapy, and it is not clear whether ustekinumab monotherapy would have achieved a similar response.