Care Programme for Patients with Ectodermal Dysplasia
Bergendal B, National Oral Disability Centre, The Institute for Postgraduate Dental Education, Jönköping, Sweden.
The objective of the consensus conference was to establish diagnostic criteria and propose a comprehensive care programme for the diagnosis hypohidrotic ectodermal dysplasia.
Materials and methods
Scandinavian specialists in different dental disciplines together with clinical geneticists and dermatologists were called as experts to the consensus conference. Before the conference, a Nordic inventory of individuals with hypohidrotic ectodermal dysplasia was made to chart the number of missing teeth in the population.
Reference: Bergendal B, Koch G, Kurol J, Wänndahl G (eds.): Consensus Conference on Ectodermal Dysplasia with special reference to dental treatment. Stockholm: Förlagshuset Gothia AB, 1998.
The overall principles for treatment that were established at the conference recommend that treatment planning be made by a multidisciplinary team of specialists and that the patient, if possible together with his or her family, be involved in the planning process.The inventory resulted in reports on 179 individuals, most of them children and adolescents. This reflects an underdiagnosis in adults which can be explained by the fact that the inventory was made by surveying dental specialists who did not seem to have adults with this diagnosis as patients. A substantial underdiagnosis of the syndrome in all age groups is considered likely. The majority of reported individuals, almost 70%, were male. The numbers of missing teeth varied from 0 to 28 (total anodontia). A general and an oral care programme for individuals with ectodermal dysplasia was worked out and adopted by the Swedish ED –Association in 2000.Hypohidrotic ectodermal dysplasia – also called Christ-Siemens-Touraine syndrome – was first described in 1848. There are more than 150 different inheritable types of ectodermal dysplasias (EDs) recognised by the combination of physical features and symptoms an affected person has and the way they are inherited. The diagnosis of an ectodermal dysplasia syndrome is generally set when two or more ectodermal organs are affected. The X-linked hypohidrotic form is the most common of the ED syndromes and has its most typical expression in males. Individuals with hypohidrotic ectodermal dysplasia often have special facial features: agenesis of teeth; dry, thin skin with no or a decreased number of sweat glands; and sparse hair. A reduced number of secretory cells in the gastrointestinal and respiratory tracts can also give rise to symptoms. Decreased salivary secretion rates have recently been described.