Ectodermal dysplasia: a narrative review of the clinical and biological aspects relevant to oral health

Ectodermal dysplasias (ED) are disorders that affect ectodermal-derived tissues during embryonic development. These disorders occur when the ectoderm, the outermost layer of embryonic tissue does not develop normally. Patients present abnormalities of two or more ectoderm-derived structures and the clinical presentation can vary greatly depending on the type a patient has. The authors compiled and provided their perspective on articles describing the classification, molecular signaling pathways, systemic and dental implications, genetic diagnosis and dental treatment considerations for patients with ED. Emphasis was placed on the main signaling pathways affecting tooth development and the relevant signs that ED patients can present including dental anomalies. Sources included original or review articles written in English that had an ED focus from PubMed and also information available in National Foundation of Ectodermal Dysplasias website. A broad and flexible narrative review is provided regarding ED which represents a diverse array of systemic symptoms that are often present with dental-related issues. The genetic diagnosis of this condition has evolved significantly during the last decade but is still an adjunct to clinical presentation. The treatment of ED involves a multidisciplinary team encompassing primary care physicians, pediatricians, nutritionists, speech therapists, dental professionals, and geneticists. Evidence from the last decade has significantly expanded our understanding of the classification and molecular signaling pathways involved in the etiology of ED. The dental professional is a critical, essential part of the team of healthcare professionals and often the first step involved in providing personalized and humanistic care and better quality of life to the patients affected by this condition.