A Huge Thoracic Aortic Aneurysm as a Rare Complication of Hyperimmunoglobulin E Syndrome.

Hyperimmunoglobulin E syndromes (HIES) are a heterogeneous group of primary immunodeficiencies sharing manifestations including recurrent lung infection, staphylococcal skin abscesses, eczema, and significantly raised serum levels of immunoglobulin E. Arterial aneurysms are among the rare vascular manifestations of HIES that may lead to devastating outcomes. We present a case of a 17-year-old girl who had a known history of HIES since childhood. She manifested a recurrent chest infection and skin rash, and it was discovered that she had a large thoracic descending aortic aneurysm, which required surgical repair to prevent devastating complications.