• Journal of the korean academy of Pediatric Dentistry
• /
• v.27 no.2
• /
• pp.222-228
• /
• 2000

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. At least 120 subtypes of ectodermal dysplasia have been reported. The disease is usually transmitted as an X-linked recessive trait in which the gene is carried by the female and manifested in the male. Hypohidrotic ectodermal dysplasia is manifested as a triad of defects that include hypohidrosis, hypotrichosis and hypodontia. The characteristic facial features consist of asteatosis, onchodysplasia, sparse and fine blond hair, prominent forehead, a depressed nasal bridge, thick everted lips. The patient may suffer from dry skin, hyperthermia and unexplained high fever as a result of the deficiency of sweat glands. This case report presents detailed procedures of rehabilitating functional and esthetic defect of a 6-year-old boy with hypohidrotic ectodermal dysplasia along with the review of relevant literatures.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.21 no.2
• /
• pp.570-576
• /
• 1994

Ectodermal dysplasia is characterized by a partial or complete lack of primary and permanent teeth, other ectodermal structures that may be affected include the skin, hair, and sweat glands. The patient with the so-called anhidrotic type of ectodermal dysplasia exhibits dry skin, lack of sweat glands, sparse eyebrows, body hair, saddle nose, and everted lips. Genetic basis of anhidrotic ectodermal dysplasia is recessive and sex-linked, being manifested chiefly in males, but this is debatable. A 6-year-old boy, with typical signs of anhidrotic ectodermal dysplasia, was presented. Prosthetic restoratoins are of great value to these patients, both from the standpoint of function and for psychologic reasons. The need for complete denture is critical during preschool periods and continues into adulthood. The following case report is an approach to the management of a patient with anhidrotic ectodermal dysplasia.

• The Journal of the Korean dental association
• /
• v.57 no.2
• /
• pp.93-99
• /
• 2019

Ectodermal dysplasia is a genetic disorder in which various clinical manifestations involve two or more of the differentiated tissues of the ectoderm. Facial deformity, which is frequently associated with ectodermal dysplasia, appears in the form of cleft lip or cleft palate, especially in the middle facial area.Cleft and tooth defects result in decreased alveolar bone development.This leads to severe skeletal incongruity. Facial features include frontal protrusion, malar bone hypoplasia, flat nose, mandibular prominence and long lower facial height. This clinical report presents treatment including orthognathic surgery of a patient with Hypohidrotic Ectodermal dysplasia with cleft palate.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.36 no.4
• /
• pp.631-639
• /
• 2009

Ectodermal dysplasia is a genetic disease which shows various congenital dysplasias in tissues differentiated from the ectoderm. As the most common type of the ectodermal dysplasia, hypohidrotic ectodermal dysplasia(HED) shows dysplasia mainly in the hair, fingernails, teeth and the skin. Symptoms are more severe in males than in females and heterozygous females are usually normal showing no symptom. The treatment for these patients differ according to individuals, but since patients can easily become depressed socially and emotionally due to a decrease in mastication and speech function caused by multiple loss of teeth as well as some aesthetic problems, an early treatment is required. In a case, with a 10 years-old boy diagnosed with HED which shows partial edentia of the maxilla, and the edentia of the mandible in the pediatric dentistry department of the Chosun University Dental Hospital, a fabrication of denture resulted in the recovery of mastication and speech function and aesthetic improvement due to an increase of the face height.

• The Journal of Korea Assosiation for Disability and Oral Health
• /
• v.13 no.1
• /
• pp.23-27
• /
• 2017

Ectodermal Dysplasia is a genetic disorder caused by the abnormal development of two or more structures derived from the ectodermal layer. As an aspect in dentistry, ectodermal dysplasia is characterized by hypodontia, conical or peg-shaped teeth, reduced salivary secretion and decreased vertical dimension. These unfavorable oral conditions make children to have difficulties with mastication, esthetics, and even in social activities. This case report presents an alternative oral rehabilitation treatment of a 4-year-old boy with ectodermal dysplasia. A removable space maintainer with artificial teeth in maxillary arch was delivered according to the initial treatment plan. However, the child failed to adapt the appliance because of his masticatory habit. Then a fixed-type space maintainer was delivered on the patient's esthetic demand and it restored function, esthetics and self-esteem of the patient. The treatment described here suggests that individually customized considerations are essential for the oral rehabilitation of a patient with ectodermal dysplasia.

• The korean journal of orthodontics
• /
• v.30 no.6 s.83
• /
• pp.739-745
• /
• 2000

Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or mote ectodermal structures. Characteristic manifestations Include scanty hair and eyebrows, pigmented and hyperkeratinized skin around the eyes and mouth, frontal bossing with prominent supraorbital ridges, nasal bridge depression and dental anomalies. Hyperthermia or unexplained high fever as a result of the deficiency of sweat glands is common medical history. Findings of intraoral structures are anodontia or oligodontia with conical crowns. Consequently, generalized spacing and loss of vertical dimension of occlusion. Interdisciplinary approach has been performed to treat a 10-year old boy with ectodermal dysplasia. Orthodontists and a prosthodontist worked together on this case, and the result was satisfactory.

• Journal of Oral Medicine and Pain
• /
• v.31 no.2
• /
• pp.121-126
• /
• 2006

Hypohidrotic ectodermal dysplasia refers to a group of disorders with the following common features : thin, sparse or absent hair, missing or peg-shaped teeth and inability to sweat adequately. Both the primary and secondary dentition are affected. Teeth may be absent (anodontia) or reduced in number (oligodontia) and abnormally shaped. In case, A 5-year-old man presented with hypohidrosis and dry skin. He had no teeth in mouth. We report a case of hypohidrotic ectodermal dysplasia with anodontia.

• The Journal of Advanced Prosthodontics
• /
• v.3 no.2
• /
• pp.96-100
• /
• 2011

An 18-year-old male presented severe hypodontia due to hypohidrotic ectodermal dysplasia was treated with Le Fort I maxillary osteotomy with simultaneous sinus floor augmentation using the mixture of cortical autogenous bone graft harvested from iliac crest and organic Bio-Oss to position the maxilla in a right occlusal plane with respect to the mandible, and to construct adequate bone volume at posterior maxilla allowing proper implant placement. Due to the poor bone quality at other sites, ridge augmentation with onlay graft was done to construct adequate bone volume allowing proper implant placement, using tissue harvested from the iliac bone. Seven implants were placed in the maxilla and 7 implants were inserted in the mandible and screw-retained metal ceramic FPDs were fabricated. The two year follow up data showed that dental implants should be considered as a good treatment modality for patients with ectodermal dysplasia.

• Journal of the korean academy of Pediatric Dentistry
• /
• v.28 no.4
• /
• pp.626-632
• /
• 2001

Ectodermal dysplasia is a hereditary disease characterized by congenital dysplasia of one or more ectodermal structures. Intraorally, common findings are anodontia or oligodontia, conical teeth, and, consequently, generalized spacing. This case presented the oral rehabilitation of a child with hypohidrotic ectodermal dysplasia. Oral rehabilitation is important from functional, esthetic, and psychologic perspectives. Due to the absence of teeth, the volume of alveolar bone and its growth are decreased, resulting in a loss of vertical dimension and protuberant lips. The treatment involved increasing the patient's vertical dimension of occlusion, fabricating a maxillary partial denture, and using magnets to help retain the mandibular partial denture. A 5-year 7-month old Korean boy was referred to the pediatric department for examination, evaluation and treatment of his disorder. we used magnets on '73 and '83 for enhanced retention of a mandibular overdenture. The magnet used in this case was the Magfit system(GC Co., Japan).

• Journal of the korean academy of Pediatric Dentistry
• /
• v.11 no.1
• /
• pp.255-259
• /
• 1984

Ectodermal dysplasia is a rare hereditary abnormality of which the outstanding characteristics are hypodontia, hypohidrosis and hypotricosis. Patient afflicted with this malformation shows a typical face resembling that of congenital-syphilis. Dental abnormality (delayed eruption, oligodontia) becomes apparent in this child and causes the parents to bring him to the dentist. Prosthetic appliances are usually indicated and he must be adjusted or be remade as the child matures.