Autoinflammatory disease, familial, Behcet-like-3

An inborn error of immunity caused by variants in the RELA gene that cause decreased RELA (NF-kB p65) protein function. The disease is characterized by chronic mucocutaneous ulcerations, variable immune deficiency, lymphoproliferation with autoimmune cytopenias, inflammatory bowel disease, and autoinflammatory manifestations such as recurrent fever and arthralgia. Affected Individuals express various combinations of clinical features.