Ectodermal Dysplasias: Orofacial Manifestations and Management

Ectodermal dysplasias (EDs) are a genetically and clinically diverse group of hereditary conditions that affect the development of ectodermal tissue derivatives including those of the face and oral cavity. Numerous genetic mutations are known to cause the EDs, and many of these genes are associated with the WNT, NFkB, and TP63 molecular pathways that are critical for the development of ectodermal tissues [1]. Multiple molecular mechanisms are involved in these different pathways altering signaling cascades and progenitor cell proliferation resulting in the spectrum of ED phenotypes observed clinically [2–8]. ED conditions are also caused by genes that are not associated with the WNT, NFkB, or TP63 pathways adding to the diversity of molecular mechanisms causing abnormal development and/or homeostasis of ectodermal tissues [1].