Abstract
BackgroundThe ectodermal dysplasias are a large and complex group of diseases.Case presentationThis article presents a case in a 37 years old female patient, referred to the dental clinic for impairment patients maintained by the University of Pernambuco. She presented typical characteristics of Christ Siemens Touraine syndrome such as alterations of the inferior members, a great number of diffuse pigmentations, poor oral hygiene, periodontal disease, oligodontia, enamel hypoplasia, including alteration in the form and size of the teeth.ConclusionThe optimal treatment for these patients should require the multidisciplinary collaborative efforts of health professionals.
Highlights
The ectodermal dysplasias are a large and complex group of diseases.Case presentation: This article presents a case in a 37 years old female patient, referred to the dental clinic for impairment patients maintained by the University of Pernambuco
As defined and classified by Freire-Maia in 1971, ectodermal dysplasias may be subdivided into two groups: First, group A disorders, which are manifested by defects in at least 2 of the 4 classic ectodermal structures, mnemonically labeled 1, 2, 3 and 4, respectively, and second, group B disorders, which are manifested by defect signs in only one of the classic ectodermal structures above mentioned in combination with a defect in a different ectodermal structure such as ears, and lips
A multidisciplinary attention should be given to syndromic patients
Summary
As defined and classified by Freire-Maia in 1971, ectodermal dysplasias may be subdivided into two groups: First, group A disorders, which are manifested by defects in at least 2 of the 4 classic ectodermal structures (trichodysplasias, dental abnormalities, nail abnormalities and dyshidrosis), mnemonically labeled 1, 2, 3 and 4, respectively, and second, group B disorders, which are manifested by defect signs in only one of the classic ectodermal structures above mentioned in combination with a defect in a different ectodermal structure such as ears, and lips. Dental care is of great psychological benefit to syndromic patients, when they have difficulties in finding dentists able to diagnose their problem and when the treatment means a financial burden for them and their families. M.L.B.V, female, 37 years old, living in the countryside of the state of Pernambuco-Brazil, home-worker, self-related white ethnicity was referred to the dental clinic for patients with special needs of the University of Pernambuco The anamnesis showed she did not smoke or drinks alcohol, had normal somatic and mental development, excellent interaction and socialization degree. At two-year follow-up, the patient's periodontal and dental health is under control
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