Ear, nose und throat manifestations of hyper IgE-syndrome: review and report of a case with successful high-dose intravenous immunoglobulin therapy

Hyperimmunoglobulin E syndrome (Hiob syndrome or Buckley syndrome) is a rar e disorder of the immune system that can show characteristic manifestations infhe head and neck. Typical symptoms are fever, recurrent urticarial rash es, lymphadenitis, and bacterial infections of the skin and various parench ymatous organs. Diagnosis is established by elevated serum IgE concentratio ns; with the absence of any signs of allergy or parasitic disease. We prese nt our clinical experiences in managing of a 29-year old woman whose hyper IgE syndrome was diagnosed initially during of the treatment of lymphatic h yperplasia of the base of the tongue although she had typical symptoms of h yper IgE syndrome for some years. High-dose intravenous immunoglobulin ther apy (IVIG) was found to be well-tolerated and effective. More than one year after a single course of immunoglobin therapy symptoms markedly improved. Current knowledge and therapeutic options in hyper IgE syndrome are discuss ed. We recommend that IVIG be considered as one of the first choices in the treatment of hyper IgE syndrome.