Abstract:The hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder characterized by high serum levels of immunoglobulin E (IgE), recurrent cutaneous and pulmonary infections, chronic dermatitis and a variety of connective tissue and skeletal abnormalities. These patients share characteristic facial appearance and many oral manifestations. We report a case of hyper IgE syndrome (HIES) also known as “Job syndrome“.
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