Ruptured thoracoabdominal aneurysm in a 27-year-old with hyper IgE syndrome.

Abstract

Hyperimmunoglobulin E syndrome is a rare primary immunodeficiency disorder defined by high serum immunoglobulin E titers and associated with characteristic infectious, dermatologic, skeletal, and dental abnormalities. We present the case of a 27-year-old man presenting acutely with a ruptured type IV thoracoabdominal aortic aneurysm. He was successfully treated by open operative repair and discharged with long-term prophylactic antibiotics. To our knowledge, this is the first such case reported in the English literature.