JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY, vol.40, no.6, 2018 (SCI-Expanded)
Background: Hyper-immunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disease characterized by recurrent infections and elevated levels of serum immunoglobulin E. usually over 2000 IU/mL. Recurrent and chronic infection of the epidermis and squamous epithelium may also be a cause of squamous cell carcinoma (SCC). SCC is rare with HIES.