Smile Building: A Case Report of Ectodermal Dysplasia Complicated by NEMO

MISSION STATEMENT: The “Smile Builders” program, a component of the American Orthodontic Society (AOS) Foundation, partners with orthodontic manufacturers and laboratories offering materials and services to our member dental professionals who donate their time to treat the disadvantaged in their communities.

NEMO is a complicated disease caused by a genetic mutation in the IKBKG gene. The official name of this gene is “Inhibitor of Kappa light polypeptide gene enhancer in B-cells, Kinase Gamma”. NEMO can involve many different cells of the body, and it often manifests in different ways in different individuals depending on the location of the mutation inside the gene. The most common clinical findings are skin and DENTAL issues and susceptibility to specific bacterial/viral infections. It is considered an immunodeficiency that affects multiple facets of the immune system. Patients often have severe infections that can affect virtually any part of the body.