Hyperimmunoglobulin-E syndrome with recurrent infection: a review of current opinion and treatment.
Hyperimmunoglobulin E (hyper-IgE) syndrome with recurrent infection is a rare idiopathic primary immunodeficiency. It consists of a severe dermatitis with recurrent abscess formation, respiratory tract infections and very high titres of serum immunoglobulin E (IgE). Hyper-IgE syndrome is also associated with skeletal abnormalities. Variability of presentation makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiencies. The aim of this article is to review the literature in order to consider the clinical findings, etiology and treatment of this syndrome.
Erlewyn-Lajeunesse
M D
MD
Allergy and Inflammation Sciences Division (Child Health), School of Medicine, University of Southampton, Southampton General Hospital, UK. mdsel@soton.ac.uk
D002648:Child; D002894:Chromosomes, Human, Pair 4; D005260:Female; D006801:Humans; D007589:Job Syndrome / Q000235:genetics / Q000473:pathology / Q000628:therapy*; D008297:Male; D012867:Skin / Q000473:pathology