Primary Immunodeficiencies With Defects in Innate Immunity: Focus on Orofacial Manifestations.

ABSTRACT: The field of primary immunodeficiencies (PIDs) is rapidly evolving. Indeed, the number of described diseases is constantly increasing thanks to the rapid identification of novel genetic defects by next-generation sequencing. PIDs are now rather referred to as "inborn errors of immunity" due to the association between a wide range of immune dysregulation-related clinical features and the "prototypic" increased infection susceptibility. The phenotypic spectrum of PIDs is therefore very large and includes several orofacial features. However, the latter are often overshadowed by severe systemic manifestations and remain underdiagnosed. Patients with impaired innate immunity are predisposed to a variety of oral manifestations including oral infections (e.g., candidiasis, herpes gingivostomatiti

SUBMITTER: Jung S 

PROVIDER: S-EPMC7314950 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature