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.
A 17-year-old male patient who was diagnosed as HIES was admitted with purulent right ear discharge. The patient had a history of eczema starting from the age of 7 months and a history of recurrent middle ear infection starting from the age of 5. Biopsy specimens were taken from the lesion in the external auditory canal, and the lesion was reported as SCC.
Patients with autosomal recessive HIES are at an increased risk for infections and malignancies. SCC should be considered in the differential diagnosis of the patients presenting with recurrent middle ear infections and immunodeficiency.
Departments of *Pediatric Allergy and Immunology
‡ENT Department, School of Medicine, Cukurova University, Adana, Turkey
D.U.A., A.H.U., M.Y., A.S.S.: involved in drafting the manuscript or revising it critically for important intellectual content. A.S.S., D.D., M.S.: made substantial contributions to conception and design, or acquisition of data. A.S.S.: gave final approval of the version to be published.
The authors declare no conflict of interest.
Reprints: Ayse S. Sasihuseyinoglu, MD, School of Medicine, Balcali Hospital, Cukurova University, 01330 Balcali/Adana, Turkey (e-mail: firstname.lastname@example.org).
Received April 17, 2017
Accepted July 12, 2017