[Hyper-IgE syndrome with ENT manifestations. Overview and case report of successful therapy with high dosage i.v. immunoglobulin].

Hyperimmunoglobulin E syndrome (Hiob syndrome or Buckley syndrome) is a rare disorder of the immune system that can show characteristic manifestations in the head and neck. Typical symptoms are fever, recurrent urticarial rashes, lymphadenitis, and bacterial infections of the skin and various parenchymatous organs. Diagnosis is established by elevated serum IgE concentrations with the absence of any signs of allergy or parasitic disease. We present our clinical experiences in managing of a 29-year old woman whose hyper IgE syndrome was diagnosed initially during of the treatment of lymphatic hyperplasia of the base of the tongue although she had typical symptoms of hyper IgE syndrome for some years. High-dose intravenous immunoglobulin therapy (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 discussed. We recommend that IVIG be considered as one of the first choices in the treatment of hyper IgE syndrome.