Aspectos clínicos y principales manifestaciones orales presentes en pacientes con Displasia Ectodérmica. Revisión sistemática.

Abstract

Introduction: Ectodermal dysplasia is a genetic disorder that causes alterations in the structures derived from the ectoderm such as skin, hair, teeth, sweat and salivary glands, it is presented in its clinical forms: hypohidrotic/anhydrotic and hydrotic. Objective: To know the clinical aspects and the main oral manifestations presented in patients with ectodermal dysplasia. Methodology: A systematic review of clinical case reports of patients with ectodermal dysplasia was performed obtaining a sample of 51 articles registering a total of 66 patients. Results: The hypohidrotic/anhydrotic form was present in 60 (90.91%) patients being the most prevalent, extraoral signs and symptoms were reported in 65 patients in which trichopathies such as sparseness of hair 52 (80%) was the most frequent; facial alterations such as prominent forehead were present in 41 (63.08%) patients; of the skin manifestations the most prevalent was dry skin in 30 (46.15%) cases; in miscellaneous hypohidrosis represented the highest figure in 56 (86.15%) individuals. The main oral signs and symptoms were conical teeth in 39 (61.90%) cases, hypodontia in 23 (36.51%) and oligodontia in 19 (30.16%). Prosthetic treatment was the most prevalent in 30 (53.52%) patients. Conclusion: The dentists should act in time and in a multidisciplinary way, according to the age and condition of the patient to maintain proper jaw growth and mitigate facial alterations. Early dental treatment complemented with psychological therapy will undoubtedly successfully improve the quality of life of these patients.