Abstract
ABSTRACT Ectodermal dysplasia refers to a hereditary disease that affects the structures derived from the ectoderm. A key feature of this syndrome is multiple missing teeth and teeth with conical shape, affecting the primary and permanent dentition. The pathognomonic facial features make patients very similar to each other. This study aims to report a case in which a 10 years old patient was diagnosed with ectodermal dysplasia during a dental visit; we also discussed the participation of dentists in the diagnostic process of the disease. Caregivers sought dental care, anxious due to several not erupted teeth, and agenesis was confirmed after the panoramic radiograph. Clinically, dry skin, hair and sparse hair, deformed and brittle nails and nose in a “saddle” also called to attention, pointing to a case of ectodermal dysplasia suspect. Thus, the patient and her household were directed to seek medical advice, confirming the diagnosis of the syndrome that supposedly also reached other family members. Since then, the rehabilitation of the patient involved a multidisciplinary treatment planning for prosthetic rehabilitation to treat the agenesis, psycho-pedagogical care support and medical intervention for treatment of hyperopia framework present. The intra-oral characteristics are the primary factors justifying the knowledge of ectodermal dysplasia by dentists, especially the pediatric dentist, who is often the first to diagnose the disease due to the complaint of agenesis reported by parents or patients.
Highlights
Ectodermal dysplasia is a syndrome which affects the ectoderm structures, such as skin, hair, teeth, nails and glands[1]
RGO, Rev Gaúch Odontol, Porto Alegre, v.65, n.2, p. 161-167, abr./jun., 2017 and knowledge in the diagnosis process is of utmost importance since ectodermal dysplasia presents a variable degree of early clinical manifestations[6].Once the diagnosis process has been established, the affected patients can be provided with a multidisciplinary rehabilitation program where self-esteem can be restored enabling
The ectodermal dysplasia syndrome comprises a complex group of disorders[7,8] with more than 200 different forms identified[9]
Summary
Ectodermal dysplasia is a syndrome which affects the ectoderm structures, such as skin, hair, teeth, nails and glands[1]. The ectodermal dysplasia syndrome comprises a complex group of disorders[7,8] with more than 200 different forms identified[9]. Even so, it is considered a rare syndrome with an estimated frequency of 1: 100,000 births[10], with male preference in a ratio of 5: 111 and a the most common forms of dysplasia are the hypohydrotic syndrome ( known as Clouston syndrome) and the anhidrotic syndrome (Christ SiemenTouraine syndrome), the hypohydrotic presents more severity. Other causes can be pointed out such as: exposure to ionizing radiation, endocrine disorders and neoplasms[2]
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