Hyper-IgE Syndrome With Epstein-Barr Virus Associated Extranodal NK/T Cell Lymphoma of Skin

Hyper-IgE syndrome, also known as Job's syndrome, is a rare primary immunodeficiency that was first described in 1966. This syndrome is associated with an increased risk for malignancies. Here, we report an 18-year-old woman who was frequently hospitalized since childhood because of recurrent pneumonia and urinary tract infection. Multiple erythematous papules and nodules with ulceration were found on her face and right forearm. A skin biopsy showed angiocentric and angiodestructive atypical lymphoid infiltration. In situ hybridization revealed latent Epstein- Barr virus-infected lymphoid cells. Accordingly, this indicates that Epstein-Barr virus infection may have induced the extranodal natural killer/T cell lymphoma in this patient with hyper-IgE syndrome.