Although the inheritancepattern of IPEX is X-linked recessive in case of mutations in FOXP3 (with no reported female patients,and asymptomatic female carriers of the gene mutation), the disease might begenetically more heterogeneous than initially presumed as a few patients withIPEX were found not to carry mutations in FOXP3 .Furthermore, in 1 family of patients that included an affected female, IPEX wassuggested to be linked to an autosomal locus [3, 7]. The gene discussed is FOXP3; the disease is immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome.