first case of x-linked recessive anhidrotic ectodermal dysplasia with immunodeficiency from iran with unusual aspergillus infection

Nemo (nf-κb essential modulator) is a regulatory factor involved in signaling pathways of innate and adaptive immune system. hypomorphic mutation of ikbkg gene on x chromosome leads to x-linked recessive anhidrotic ectodermal dysplasia with immunodeficiency. the affected boys presented developmental phenotype with hypotrichosis, hypohidrosis; hypodontia with conical incisors; and susceptibility to pyogenic bacteria, mycobacteria, and viruses. most of them have impaired antibody response to polysaccharide antigens. here, we presented the case report of 7 years old iranian boy with nemo-deficiency and unusual aspergillus infection.