Hyperimmunoglobulin E syndrome is a Primary Immuno-deficiency Syndrome (PIDS) characterized by high serum immunoglobulin E, eczema rash and recurrent sinopulmonary and skin infections. In this report, we present a case of squamous cell carcinoma of the lower lip in a young patient with Hyper IgE syndrome. The patient had initially developed a chronic non-healing ulcer and a biopsy done revealed a squamous cell carcinoma. Immunodeficiency has been recognized as an essential risk factor for cancer through immune dysregulation and defective immunosurveillance.
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