Behçet-like intestinal disease in a patient with trisomy 8-associated myelodysplastic syndrome (TRIAD)

A woman in her late 60s from South India presented with a year-long history of recurrent oral and genital ulcers, abdominal pain and low-grade fever. Evaluation showed ileocaecal ulcers, cytopenias and Trisomy 8-positive myelodysplastic syndrome (MDS). The constellation of mucocutaneous and gastrointestinal Behçet-like features with clonal haematopoiesis led to the diagnosis of Trisomy 8-Associated Autoinflammatory Disease (TRIAD), also known as Behçet-like intestinal disease with MDS. She initially responded to corticosteroids and azacitidine, but relapsed whenever steroids were tapered below 10 mg/day, reflecting a refractory, steroid-dependent course. Management targeted the underlying high-risk MDS with azacitidine, which also exerted potent anti-inflammatory effects. Other advanced therapies, including biologic agents used for primary Behçet's disease, were not considered appropriate due to the patient's high-risk MDS and cytopenias. This case underscores the importance of recognising TRIAD in South Asia, where overlap with tuberculosis and inflammatory bowel disease may delay diagnosis, and highlights azacitidine's dual role and haematopoietic stem cell transplantation as the only definitive option. This case was managed between 2023 and 2024, raising the possibility of concurrent or prior SARS-CoV-2 infection contributing to the observed immune dysregulation, a factor increasingly recognised in autoinflammatory conditions.