Hereditary Ectodermal Dysplasia Research Articles

Hereditary Ectodermal Dysplasia in Two Identical Siblings

Hereditary Ectodermal Dysplasia in Two Identical Siblings

The Early Prostehtic Management Of Hypohidrotic And Anhidrotic Ectodermal Dysplasia: About Two Clinical Cases

Ectodermal dysplasia is a hereditary disorder. Patients with ectodermal dysplasia have ectodermally-derived organs disorders which can affect teeth, nails, hair and sweat glands. Disorders of sweat gland function can be classified into two major groups: hypohidrotic or anhidrotic. Oral findings in patients with ectodermal dysplasia (ED) include markedly reduction in number of teeth (oligodontia or hypodontia), malformed and widely spaced conical-shaped teeth, and underdeveloped alveolar ridges. Other dominant clinical features can be found like very thin hair. The goal of the article is to present the prosthetic management particularities of two patient with hereditary ectodermal dysplasia.

Prosthodontic rehabilitation of a hypohydrotic ectodermal dysplasia patient with extracoronal attachments: A case report

Prosthodontic rehabilitation in a patient with hereditary ectodermal dysplasia (ED) is often challenging due to compromised intraoral supporting structures. The recent advances in dental materials and improved techniques provide an array of options in restoring both the aesthetics and function of the stomatognathic system, especially in complicated scenarios. The literature search provided diversified modalities of treatment approaches for individuals affected with ED. This article describes the use of an extra coronal attachment in the prosthodontic management of a hypohydrotic ectodermal dysplasia patient presenting with severe oligodontia.

A new dimension in the dental rehabilitation of hereditary ectodermal dysplasia

Partial or complete anodontia is one of the major dental findings in a patient of Hereditary Ectodermal Dysplasia (HED) and is diagnosed at a young age when the parents are concerned about the child’s functional needs and esthetic appearance. The oral rehabilitation of these cases is often very challenging. Removable partial and complete dentures are most commonly used treatment modalities for such patients. Although implants and implant supported dentures can be considered, age is one of the compromising factors for placement of implants in young patients due to the possibility of impending growth of jaws. We present here a case of 3 and a half year old male patient of HED treated successfully with implants. With a long term follow up of 6 years, we achieved excellent functional and esthetic results thereby boosting the self-confidence of the child. Keywords: Hereditary ectodermal dysplasia, Anodontia, Dental implants.

Ectodermal Dysplasia—A Case Report

AbstractEctodermal dysplasias (EDs) include large group of syndromes that are clinically and genetically heterogeneous and are identified by anomalies in structures of ectodermal origin. Hereditary ectodermal dysplasia is a condition associated with defect of structures originating from ectoderm and is typically inherited as cross-linked recessive trait, more pronounced in males than in females. The frequency of different ectodermal dysplasias in a given population is highly variable. It is estimated to be 7 in 1,000 births. This case report is presented with an objective to show two patients, siblings, with ED. Both presented with classic symptoms of anodontia, hypohidrosis, and hypotrichosis.

Prosthetic Rehabilitation in a Child with Hereditary Ectodermal Dysplasia

Hereditary ectodermal dysplasia is a disease linked to the X recessive chromosome that affects the development of tissues derived from the ectoderm. The main clinical manifestations include hypohidrosis (inability to sweat), hypotrichosis (sparse hair) and hypodontia (lack teeth). This work presents a clinical case of a 6-year old male child who had been diagnosed with hereditary ectodermal dysplasia. The treatment consisted of the fabrication of a mandibular and maxillary complete denture to improve his esthetic, functional and psychological conditions.

A Rare Case Report of Non- Hereditary Ectodermal Dysplasia with Impacted Teeth in Maxilla and Mandible in 18 Year Old Male

A Rare Case Report of Non- Hereditary Ectodermal Dysplasia with Impacted Teeth in Maxilla and Mandible in 18 Year Old Male

Hereditary ectodermal dysplasia with hypohydrosis: A report of two female siblings with autosomal recessive type inheritance

The Journal is the primary organ of Continuing Paediatric Medical Education in Sri Lanka. The journal also has a website. Free full text access is available for all readers.The Sri Lanka Journal of Child Health is now indexed in SciVerse Scopus (Source Record ID 19900193609), Index Medicus for South-East Asia Region (IMSEAR), CABI (Centre for Agriculture and Bioscience International Global Health Database), DOAJ and is available in Google, as well as Google Scholar.The policies of the journal are modelled on the Committee on Publication Ethics (COPE) Guidelines on Principles of Transparency and Best Practice in Scholarly Publishing. Sri Lanka Journal of Child Health is recognised by the International Committee of Medical Journal Editors (ICMJE) as a publication following the ICMJE Recommendations.

Nectins and nectin-like molecules in development and disease.

Nectins and nectin-like molecules (Necls)/Cadms are Ca(2+)-independent immunoglobulin superfamily cell adhesion molecules, expressed in most cell types. Nectins mediate not only homotypic but also heterotypic cell-cell adhesion, in contrast to classic cadherins which participate only in homophilic adhesion. Nectins and Necls function in organogenesis of the eye, inner ear, tooth, and cerebral cortex and in a variety of developmental processes including spermatogenesis, axon guidance, synapse formation, and myelination. They are also involved in various diseases, such as viral infection, hereditary ectodermal dysplasia, Alzheimer's disease, autism spectrum disorder, and cancer. Thus, nectins and Necls are crucial for both physiology and pathology. This review summarizes recent advances in research on these cell adhesion molecules in development and pathogenesis.

Complete Denture Treatment In A Child With Ectodermal Dysplasia: A Clinical Report

The process of rehabilitation in hereditary ectodermal dysplasia is challenging because it takes a long duration involving multiple prostheses. If the patient is very young and completely edentulous the clinical methods have to be stretched to unimaginable proportions. A case is reported where complete dentures had to be fabricated at an early age of 4 years. The most challenging task was the fabrication of a face bow. Teeth also had to be characterized to suit the child.

Anhydrotic Ectodermal Dysplasia With Pregnancy- A Case Study

Hereditary Ectodermal Dysplasia is an inherited disorder involving skin, teeth, hair and nails. Hypohidrotic ectodermal dysplasia or Anhydrotic ectodermal dysplasia is the most common syndrome among this large group of heriditory disorders. Hypohydrosis/anhydrosis, hypotrichosis and hypodontia constitute the main symptoms of the syndrome. The case of a 28 year old lady with Anhidrotic ectodermal dysplasia and pregnancy with positive family history is described. Bangladesh Journal of Medical Science Vol. 12 No. 02 April’13 Page 235-237 DOI: http://dx.doi.org/10.3329/bjms.v12i2.14980

Hereditary ectodermal dysplasia: Report of 11 patients from a family

Hereditary Ectodermal Dysplasia is an inherited disorder commonly involving skin, teeth, hair, and nails. We have observed ectodermal dysplasia (EDs) in 11 individuals over two generations in one family. Smooth, dry, thin skin was seen in most affected individuals. All had fine, slow-growing scalp hair and body hair and some had sparse eyebrows and short eyelashes. Nearly all showed decrease in sweating. Severe teeth abnormalities were seen in all patients and fingernail abnormalities were not so severe but toenail abnormalities were seen in all patients. No other abnormalities were seen in affected individuals in this family. It is very rare to find such a large family having ectodermal dysplasia.

Functional esthetic rehabilitation of a 7-year-female patient with hereditary ectodermal dysplasia using flexible denture

Functional esthetic rehabilitation of a 7-year-female patient with hereditary ectodermal dysplasia using flexible denture

Hereditary ectodermal dysplasia: A retrospective study

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.

A Study of Cases of Hereditory Ectodermal Dysplasia - A Rare Dental Anomaly

Hereditary ectodermal dysplasia is a group of disorder running in the family where more than one manifestation occurs involving skin, nail, hair, glands and teeth In the present study, five cases were detected in district of Bangalore,Karnataka and studied in detail. Out of them three were girls and two boys showing manifestation dysplasia of teeth, skin & sweat glands between the age groups of 5 years to 14 years of age. There are three girls between 5 to 18 years showing oligodentia (0.13%) in 2 girls and anodentia in one girl (0.67%) associated with periorbital wrinkling and mild mid facial hypoplasia.The other 2 were boys between 8years and 15 years of age showed oligodentia, anhydosis brittle nails with vertical ridges, and 15 years old boy also showed periorbital wrinkling. The mothers of these five patients were also studied. Consanguity along with heredity and hypertension has played a vital role in the development of ectodermal dysplasia These 5 cases were compared and correlated with available literatures.

Prosthodontic Rehabilitation of Hereditary Ectodermal Dysplasia in an 11-Year-Old Patient with Flexible Denture: A Case Report

Hereditary ectodermal dysplasia is a rare group of inherited disorders characterized by aplasia or dysplasia of two or more tissues of ectodermal origin such as hair, nails, teeth, and skin. The dental characteristics of this syndrome include anodontia or hypodontia of the primary and/or permanent teeth, hypoplastic conical teeth, and underdevelopment of the alveolar ridges. The options for a definitive treatment plan include fixed, removable or implant-supported prostheses, singly or in combination. This clinical report describes the prosthetic rehabilitation of an 11-year-old boy with hereditary ectodermal dysplasia. Maxillary flexible removable partial denture and mandibular conventional complete denture were fabricated to establish an acceptable masticatory function, speech, and esthetics for the patient.

Prosthodontic rehabilitation of patient with hereditary ectodermal dysplasia

Hereditary ectodermal dysplasia is X-linked disorder, characterized by the defective formation of ectodermal structures of the body, e.g. skin, nails, sweat glands, sebaceous glands, and hair follicles. Three most outstanding features of this disorder are hypohydrosis, hypotrichosis, and complete or partial anodontia, which involves both deciduous and permanent dentition. This case report describes the prosthodontic management of a young male patient affected by this disorder.A 30-year-old male patient reported to VSPM's Dental College and Hospital, Nagpur, for the complaint of missing teeth in the upper and lower jaws. He had problem with mastication and esthetics. The permanent teeth erupted in the upper arch and were lateral incisors, canines, and first molars on both right and left sides of the arch. The anterior teeth were small and conical in shape. Over-retained deciduous second molars were present in the upper arch. In the lower arch, only a single first molar was present on the left side and the remaining alveolar ridge was resorbed.After routine dental investigations such as diagnostic casts and radiographs, the maxillary arch anterior as well as the posterior teeth were prepared to receive fixed partial dentures, and in the mandibular arch, overdenture was planned.Initially, the patient was unhappy with his looks. Also because of the inability in mastication, his general health was impaired in the young age. After prosthodontic management with such disorder, the young patient's facial esthetics was improved a lot and his morale was boosted with confidence.

Karyotyping, dermatoglyphic, and sweat pore analysis of five families affected with ectodermal dysplasia

Background:Hereditary ectodermal dysplasia is a genetic recessive trait characterized by hypohydrosis, hypotrichosis, and hypodontia. The affected individual show characteristic physiognomy like protruded forehead, depressed nasal bridge, periorbital wrinkling, protruded lips, etc. There is marked decrease in sweat and salivary secretion. Due to skin involvement palm and sole ridge patterns are disrupted.Aim:In this study an attempt has been made to classify the affected members according to the degree of penetrance by pedigree analysis and also study karyotyping for cytogenetics, dermatoglyphic analysis for the various ridge patterns and variations in the number of sweat glands by sweat pore analysis in affected individuals.Materials and Methods:A total of five families who were affected with ectodermal dysplasia were considered. Pedigree analysis was drawn up to three generation by obtaining history. Dermatoglyphics and sweat pore analysis was done by obtaining palm and finger print impression using stamp pad ink. Karyotyping was done by collecting 3–5 ml peripheral blood. Karyotyping was prepared using lymphocyte culture. Chromosomes were examined at 20 spreads selected randomly under ×100 magnification. Results were analyzed by calculating mean values and percentage was obtained.Results:Karyotyping did not show any abnormalities, dermatoglyphic analysis and sweat pore counts showed marked variations when compared with normal. Moreover, pedigree analysis confirmed the status of the disease as that of the recessive trait.Conclusion:Large number of affected patients needs to be evaluated for dermatoglypic analysis. Genetic aspect of the disease needs to be looked into the molecular level in an attempt to locate the gene locus responsible for ectodermal dysplasia and its manifestation.

Hypohidrotic (anhidrotic) ectodermal dysplasia in female twins

Autosomal recessive hereditary ectodermal dysplasia (HED) has not been described in sub-Saharan Africa. It is acknowledged to be rarer than the occasionally reported x-linked and autosomal dominant variants. We report a pair of Nigerian female twins with family history and clinical features suggestive of recessive HED, thereby showing the existence of this rare form in sub-Saharan Africa.

Prosthetic management of an 11-year-old patient with hereditary ectodermal dysplasia and partial anodontia – a case report.

Objective: To illustrate the problems of partial anodontia and replacement of missing teeth and bone in patients with ectodermal dysplasia.Method: The illustrative case is an 11year old boy with tuberculoid shaped 11, 21 and 16 as the teeth present in the upper arch with anodontia of lower arch. Result: Upper removable partial denture and lower removable complete denture was fabricated and fitted satisfactory. Conclusion: The psychological and social embarrassment suffered by children with ectodermal dysplasia associated with missing teeth can be greatly improved with early prosthetic rehabilitation with removable prostheses. Further management with fixed prosthesis may be undertaken as they grow older.Key words: Ectodermal dysplasia, Prosthetic treatment.