The IFAP syndrome is a rare X-linked recessive inheritance disorder with defect of the MBTPS2 gene defined by the triad of follicular ichthyosis, alopecia, and photophobia. A total of 40 cases has been reported, but no correlation with Hodgkin lymphoma has been reported yet. A 3.5-year-old boy was diagnosed with IFAP syndrome confirmed by Next Generation Sequencing. He was on regular follow-up when he developed prolonged fever and lymphadenopathy. His lymph node biopsy showed Hodgkin lymphoma with mixed cellularity subtype. This case is the first report on IFAP syndrome associated with malignancy. IFAP syndrome could be a risk factor in developing malignancy.
*Department of Pediatrics, Division of Pediatric Hematology and Oncology
†Neonatal Research Center, Shiraz University of Medical Sciences, Shiraz, Iran
The authors declare no conflict of interest.
Reprints: Mahdi Shahriari, MD, Department of Pediatrics, Division of Pediatric Hematology and Oncology, Shiraz University of Medical Sciences, Shiraz 71937-11351, Iran (e-mail: firstname.lastname@example.org).
Received December 8, 2016
Accepted May 22, 2017