Hyperimmunoglobulin E-recurrent infections Jobs syndrome

The inflammatory response in HIE patients is strikingly abnormal, despite the large local infections that occur. Neutrophils are present only at low numbers in these sites. It has been suggested that too few neutrophils arrive too late to deal with the pathogens, which thus have the opportunity 

This is a rare, inherited disorder characterised by recurrent and, often, life-threatening infections. This dysfunction results from absent or very low expression of the glycoprotein family, LFA-1, CR3 and gp 150,95, which function in leukocyte adhesion ( 3.9). These adhesion molecules are normally responsible for various functions, such as the following  

---