Ectodermal Dysplasia with Anodontia: A Report of Two Cases

Bani, Mehmet; Tezkirecioglu, Ali Melih; Akal, Neşe; Tüzüner, Tamer

doi:10.1055/s-0039-1697832

Cited by 30 publications

(39 citation statements)

References 10 publications

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“…[11415] They represent a large and complex group of diseases comprising more than 170 different clinical conditions. [21516] There are two major types of ED depending on the number and functionality of the sweat glands; Hypohydrotic and Hydrotic. [1517] Hypohydrotic type, also called as anhydrotic type, is the most common ED (80%) and is often inherited as an X-linked disorder (XLEDA).…”

Section: Introductionmentioning

confidence: 99%

“…[21516] There are two major types of ED depending on the number and functionality of the sweat glands; Hypohydrotic and Hydrotic. [1517] Hypohydrotic type, also called as anhydrotic type, is the most common ED (80%) and is often inherited as an X-linked disorder (XLEDA). [14] It is characterized by the classical triad of hypodontia, hypohidrosis and hypotrichosis with characteristic dysmorphic facial features.…”

Section: Introductionmentioning

confidence: 99%

“…It is also termed as Christ-Siemens-Tauraine syndrome. [1015] The hydrotic form usually has normal sweat glands and the condition is inherited as autosomal dominant. This form also affects teeth, nails and hair.…”

Section: Introductionmentioning

confidence: 99%

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Hereditary ectodermal dysplasia: A retrospective study

More¹,

Bhavsar²,

Joshi³

et al. 2013

J Nat Sc Biol Med

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Background:Ectodermal dysplasia (ED) is a group of rare, inherited disorders characterized by sparse hair, missing teeth and inability to sweat.Objective:To review and analyze cases of ED with an emphasis on clinical manifestations and parent's marriage history.Methodology:The present retrospective study was conducted by assessing the clinical records of nineteen cases of ED, available in the archives of the department; for age, gender, family history of consanguineous marriage and clinical manifestations.Results:It was observed that ED was more prevalent in males, with a ratio of 1.7:1. The hypohydrotic type was more common (78.95%) than hydrotic type (21.05%). The marriage history of parents revealed that 66.67% had consanguineous marriage and had 68.42% offspring's affected with ED; whereas 33.33% had history of non-consanguineous marriage and had 31.58% offspring's affected with ED. The clinical manifestations observed were- dry skin(94.74%); scaly skin(42.11%); sparse hair on scalp, eyebrows and eyelashes(100%); frontal bossing(63.18%); saddle nose (57.89%); hypertelorism (47.37%); nail abnormality(52.63%); normal sweat glands(21.05%); abnormal sweat glands(78.95%); hypoplastic maxilla(52.63%); protuberant lips (57.89%); palmo-plantar keratosis(21.05%); wrinkled & hyper pigmented facial skin(84.21%); partial anodontia(94.74%); conical shaped teeth(84.21%); high arched palate(68.42%); thin alveolar bone(100.00%); taurodontism(21.05%) and cleft lip & cleft palate(05.26%). The number of teeth present in all the cases ranged from 0 to 19.Conclusion:ED patients suffer from social problems and poor psychological and physiological development as a result of unacceptable esthetics and abnormal function of orofacial structures. Oral rehabilitation thus becomes mandatory, although it is often difficult; particularly in pediatric patients.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Hereditary ectodermal dysplasia: A retrospective study

More¹,

Bhavsar²,

Joshi³

et al. 2013

J Nat Sc Biol Med

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show abstract

“…Depending on the severity of clinical manifestations, Christ-Siemens-Touraine syndrome can be classified as either hypohidrotic or anhidrotic ectodermal dysplasia 7. It has full expression only in men.…”

Section: Discussionmentioning

confidence: 99%

Familial ectodermal dysplasia: a peers' agony

Hegde

Kashyap²,

Nair

et al. 2013

Case Reports

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Ectodermal dysplasias include a various group of inherited disorders which share primary defect in the development of two or more tissues of embryonic ectodermal origin. Though there are many subtypes, ectodermal dysplasias are mainly hidrotic ectodermal dysplasia and hypohidrotic ectodermal dysplasia, among which the most common variety is X linked hypohidrotic ectodermal dysplasia. We report a rare case of X linked hypohidrotic ectodermal dysplasia occurring in a family with various skin, hair and oral abnormalities.

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“…Shaw describes the need for these to restore vertical dimension and prevent them undesirable protruding lips secondary to over closure, thereby improving the profile. Ideally, a restored dentition should be in place before the child attends primary school as oral rehabilitation has a major psychological impact on the patient’s self esteem and facilitating social acceptance 12 13…”

Section: Discussionmentioning

confidence: 99%

Prosthodontic management of patients with Christ-Siemens-Touraine syndrome

Shigli¹,

Sarkar²

2012

Case Reports

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Christ-Siemens-Touraine syndrome also known as anhidrotic ectodermal dysplasia. Ectodermal dysplasia is a hereditary disorder characterised by developmental dystrophies of ectodermal derivatives. It is characterised by triad of signs comprising sparse hair, abnormal or missing teeth and inability to sweat. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. The case of three children with anhidrotic ectodermal dysplasia with partial anodontia is presented. Dental, oral and physical features were taken into consideration in diagnosis and different treatment modalities for this patient. Clinical management consisted of removable partial prosthesis in maxillary arch and complete denture prosthesis in mandibular arch. The main aim of the treatment was to improve psychological development and to promote better functioning of the stomatognathic system.

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Ectodermal Dysplasia with Anodontia: A Report of Two Cases

Cited by 30 publications

References 10 publications

Hereditary ectodermal dysplasia: A retrospective study

Hereditary ectodermal dysplasia: A retrospective study

Familial ectodermal dysplasia: a peers' agony

Prosthodontic management of patients with Christ-Siemens-Touraine syndrome

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