Causes of death in hyper-IgE syndrome

AF Freeman, DE Kleiner, H Nadiminti, J Davis… - Journal of allergy and …, 2007 - Elsevier
BACKGROUND: Hyper-IgE syndrome (HIES) is characterized by recurrent pyogenic
infections, eczema, increased serum IgE levels, and a variety of connective tissue and
skeletal system abnormalities. Little has been published regarding the causes of death in
these patients or pathologic findings. OBJECTIVE: To identify the cause of death in patients
with HIES and to describe pathologic findings in fatal HIES. METHODS: We reviewed the
medical records and autopsy slides of 6 patients with HIES with autopsies performed at our …