Ectodermal dysplasia: a clinical overview for the dental practitioner

Abstract

The term ectodermal dysplasia (ED) is used to describe a group of rare congenital disorders characterized by abnormalities of two or more ectodermal structures such as the skin, hair, nails, teeth and sweat glands. This paper will give an overview of the aetiology of ED and describe the manifestations and dental management of this condition. In particular, the important role of the dental practitioner in the identification and management of patients with ED will be highlighted. CPD/Clinical Relevance: Dental practitioners should be aware of the oral features of ectodermal dysplasia and be able to make timely referrals and provide appropriate continuing care for these patients.

Dental-craniofacial manifestation and treatment of rare diseases

“…7 The abnormality in the development of tooth buds frequently results in congenital hypodontia (both primary and permanent dentitions) and/or changes in tooth morphology or size, such as peg-shaped or pointed teeth, taurodontism and enamel defects, including hypoplasia. 9 The degree of tooth missing is always in the mild to moderate range, and a wide variation is observed regarding which teeth are missing; however, the most frequently reported missing teeth are the first molars, upper central incisors and canines 10–14 (Table 1). Accordingly, composite restorations or crowns are almost always necessary for children as early as 2 years of age, and multiple denture replacements are often needed as the child grows, with dental implants providing a potential option in adolescence when the jaw is fully grown.…”

Dental Rehabilitation of a Child with Ectodermal Dysplasia: A Case Report

“…The enamel may be softer and thinner than normal which leads to increase in cavities; also, the enamel could contain pitted surfaces. 7 Saliva production may be reduced in some forms of ED leading to dry mouth which might increase the risk for tooth decay. 7 The shape of the teeth may be globe-shaped and smaller than averages and often are described as being conical and tapered.…”

“…7 Saliva production may be reduced in some forms of ED leading to dry mouth which might increase the risk for tooth decay. 7 The shape of the teeth may be globe-shaped and smaller than averages and often are described as being conical and tapered. In some ED, the pulp chambers are larger than normal (taurodontia).…”

Etiología, manifestaciones clínicas y estudio por imágenes de la displasia ectodérmica: Una revisión de la literatura

“…El tratamiento debe realizarse en forma individual tan pronto como sea posible, dependiendo de la edad, la agenesia dental, el grado de malformación de los dientes, el crecimiento y desarrollo del sistema estomatognático y la motivación del paciente, dirigido a mantener la dentición presente y las crestas alveolares, ya que estas estructuras tendrán que soportar prótesis toda la vida (10)(11)(12).…”