Prosthetic rehabilitation of a hypohidrotic ectodermal dysplasia patient: A case report

Aim: Ectodermal dysplasia is a rare hereditary disease that arises from a developmental disorder of 2 or more ectoderm-derived tissues. Ectodermal dysplasia is seen in 3 different types: anhidrotic, hypohidrotic, and hidrotic. Its anhidrotic and hypohidrotic types are the most common. This study presents the intraoral findings and dental treatment approach of a case diagnosed with hidrotic ectodermal dysplasia that demonstrates the typical characteristics of the disease, such as anodontia, hypohydrosis (reduced sweating), hypotrichosis (sparse hair), and loss of vertical dimension. Methodology: A 5-year-old male patient presented to the clinic of the Prosthetic Dental Treatment Department of the School of Dentistry at Dicle University on 25.10.2020 with complaint of missing teeth. A genetic analysis conducted in 2016 showed that he was a homozygous carrier of the p.Cys148Arg (c.442 T>C) mutation on the 5th exon of the ectodysplasin-A receptor (EDAR) gene. The mutation detected in the patient was associated with ectodermal dysplasia. An extraoral clinical examination revealed sparse hair, eyebrows, and eyelashes; soft, smooth, and dry skin; thin, linear wrinkles around the eyes and the lips; drooping, thickened lips; a sunken nose; fractured nails; hyperthermia due to lack of sweat glands; hyperkeratosis in the skin and soles of the feet; 2 nipples on one side of the chest; and reduced vertical facial height. An intraoral examination revealed anodontia; there were no teeth on the maxilla or the mandible and no radiographically identified tooth germ. Dry mouth due to a lack of sufficient saliva was another finding. Conclusion: In this case report, in the presence of anodontia, a removable total prosthesis, which is a non-invasive treatment option, was applied. Production of endosseous implants was postponed for a later time following the patient’s growth and development. How to cite this article: Gökçe D, Ayna E, Seyfioğlu Polat Z. Prosthetic rehabilitation of a hypohidrotic ectodermal dysplasia patient: A case report. Int Dent Res 2021;11(Suppl.1):292-8. https://doi.org/10.5577/intdentres.2021.vol11.suppl1.43 Linguistic Revision: The English in this manuscript has been checked by at least two professional editors, both native speakers of English.

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Hypohidrotic ectodermal dysplasia - a case report / Hipohidrotska ektodermalna displazija – prikaz slučaja

Serbian Journal of Dermatology and Venerology ◽

10.2478/v10249-011-0042-8 ◽

2011 ◽

Vol 3 (3) ◽

pp. 109-112

Author(s):

Svetlana Popadić ◽

Andreja Vujanac ◽

Biljana Arsov ◽

Petar Ivanovski ◽

Miloš Nikolić

Keyword(s):

Case Report ◽

Growth And Development ◽

Ectodermal Dysplasia ◽

Abnormal Development ◽

Sweat Glands ◽

Facial Features ◽

Palmoplantar Keratoderma ◽

Hypohidrotic Ectodermal Dysplasia ◽

Ectodermal Dysplasias ◽

Sparse Hair

Abstract Ectodermal dysplasias are a large group of disorders characterized by developmental dystrophies of one or more ectodermal structures. Hypohidrotic ectodermal dysplasia is a rare genodermatosis associated with abnormal development of sweat glands, teeth, and hair. Its incidence is 1:100.000 newborns. The full expression of X-recessive forms are only seen in males, while female heterozygotes are moderately or very slightly affected. The disease is characterized by sparse hair, oligodontia, and reduced or absent sweeting, light hair, distinctive facial features, palmoplantar keratoderma. We report an 11-year-old boy with hypohidrotic ectodermal dysplasia. Despite extensive skin, teeth and hair manifestations, his physical and psychomotor growth and development were normal.

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Rehabilitation of ectodermal dysplasia patient with a telescopic denture in the maxilla and mandibular implant assisted over-denture: a case report

10.22541/au.163299155.56550462/v1 ◽

2021 ◽

Author(s):

Heba Alajami ◽

Jamal Saker

Keyword(s):

Case Report ◽

Ectodermal Dysplasia ◽

Clinical Report ◽

Prosthetic Rehabilitation ◽

Hypohidrotic Ectodermal Dysplasia

Hypohidrotic ectodermal dysplasia is a heritable disease, characterized by hypodontia, hypotrichosis, and anhidrosis. This clinical report demonstrates prosthetic rehabilitation of a patient complaining of impaired mastication and the odd appearance of her jaws. Maxillary telescopic-overdenture and implant-supported mandibular prostheses were fabricated to improve mastication, speech, and aesthetics of the patient.

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Persistent nasal crusting due to hypohidrotic ectodermal dysplasia

The Journal of Laryngology & Otology ◽

10.1017/s0022215100133687 ◽

1996 ◽

Vol 110 (4) ◽

pp. 379-382 ◽

Cited By ~ 12

Author(s):

A. H. Al-Jassim ◽

A. C. Swift

Keyword(s):

Respiratory Tract ◽

Ectodermal Dysplasia ◽

Sweat Glands ◽

Specific Reference ◽

Nasal Discharge ◽

Upper Respiratory Tract ◽

Hypohidrotic Ectodermal Dysplasia ◽

Sparse Hair ◽

Tract Infections ◽

Clinical Awareness

AbstractHypohidrotic ectodermal dysplasia is an hereditary condition of the ectodermal tissues which may escape recognition because of lack of clinical awareness due to its rarity. Otorhinolaryngological features of this syndrome include chronic respiratory tract infections, persistent foul-smelling nasal discharge and crust formation, and hearing problems.The condition is usually an X-linked recessive disease affecting mainly ectodermal tissue, although nonectodermal tissue may also be affected. The notation ‘ectodermal’ is used because ectodermal tissues are always involved. The syndrome is characterized by complete or partial absence of sweat glands, sparse hair growth, absent or deformed peg teeth, sparse sebaceous glands, occasional absence of salivary and lacrimal glands, scanty mucous glands and deficient cilia.We describe two children with this disorder with specific reference to the effect on the upper respiratory tract.

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Prosthetic rehabilitation with fixed prosthesis of a 5-year-old child with Hypohidrotic Ectodermal Dysplasia and Oligodontia: a case report

Journal of Medical Case Reports ◽

10.1186/s13256-019-2268-4 ◽

2019 ◽

Vol 13 (1) ◽

Cited By ~ 1

Author(s):

Reema AlNuaimi ◽

Mohammad Mansoor

Keyword(s):

Quality Of Life ◽

Ectodermal Dysplasia ◽

Genetic Disorder ◽

Middle Eastern ◽

Sweat Glands ◽

Aesthetic Outcome ◽

Prosthetic Rehabilitation ◽

Hypohidrotic Ectodermal Dysplasia ◽

Fixed Prosthesis

Abstract Background Ectodermal dysplasia is a rare genetic disorder that affects ectodermally derived structures, including teeth, nails, hair, and sweat glands. Hypohidrotic ectodermal dysplasia is the most common type, with oligodontia being the most striking dental feature. Prosthetic rehabilitation in children with ectodermal dysplasia is an important step toward improving their overall quality of life. The fixed prosthesis has the advantages of being more stable in the mouth with good child compliance and a good aesthetic outcome. Case presentation Our patient was a 5-year-old Middle Eastern boy with oligodontia caused by ectodermal dysplasia. He was managed by fabrication of an upper functional space maintainer and a lower fixed partial denture to restore occlusion, masticatory function, aesthetics, and overall quality of life. Conclusions The use of the fixed prosthesis in children is a new and evolving treatment modality that resolves many of the issues caused by removable prostheses. It accommodates jaw growth in the mandible, reduces the need to remake the prosthesis, and has an overall better aesthetic outcome.

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Prosthetic rehabilitation of an adolescent with hypohidrotic ectodermal dysplasia with partial anodontia: Case report

Journal of Indian Society of Pedodontics and Preventive Dentistry ◽

10.4103/0970-4388.44041 ◽

2008 ◽

Vol 26 (4) ◽

pp. 177 ◽

Cited By ~ 7

Author(s):

S Kaul ◽

R Reddy

Keyword(s):

Case Report ◽

Ectodermal Dysplasia ◽

Prosthetic Rehabilitation ◽

Hypohidrotic Ectodermal Dysplasia

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Rapp-Hodgkin Syndrome: Clinical and Dental Findings

Journal of Clinical Pediatric Dentistry ◽

10.17796/jcpd.34.1.kr015833p1qg6873 ◽

2009 ◽

Vol 34 (1) ◽

pp. 71-75 ◽

Cited By ~ 3

Author(s):

Gul Tosun ◽

Ulku Elbay

Keyword(s):

Cleft Palate ◽

Ectodermal Dysplasia ◽

Dental Treatment ◽

Sweat Glands ◽

Oral Manifestations ◽

Steel Wool ◽

Rare Form ◽

Dental Rehabilitation ◽

Delayed Eruption ◽

Sparse Hair

Rapp-Hodgkin syndrome is a rare form of ectodermal dysplasia involving the hair, eyes, sweat glands, nails,teeth and palate. This syndrome is characterized by stiff, sparse hair with the appearance of steel wool,sparse eyebrows and lashes, cleft palate, absence of lacrimal punctae, epiphora, a decreased number of sweat glands, and dystrophic nails. Cleft palate, hypodontia, abnormal tooth shape, multiple caries, delayed eruption of teeth are the main oral manifestations. In this paper we describe the clinical and dental findings of this syndrome in a 7 year old girl referred to the dental clinic because of severe tooth ache. The dental treatment included root canal treatment, composite restorations, extractions and removable prostheses. This paper highlights features of Rapp-Hodgkin Syndrome and its dental rehabilitation.

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Treatment Considerations for a Patient with Hypohidrotic Ectodermal Dysplasia: A Case Report

The Journal of Contemporary Dental Practice ◽

10.5005/jcdp-9-3-128 ◽

2008 ◽

Vol 9 (3) ◽

pp. 128-134 ◽

Cited By ~ 3

Author(s):

Dilek Tüfekcioğlu ◽

Chenkhan Bal ◽

Bilge Turhan Bal

Keyword(s):

Case Report ◽

Behavioral Problems ◽

Ectodermal Dysplasia ◽

Young Patients ◽

Hair Follicles ◽

Sweat Glands ◽

Clinical Report ◽

Hypohidrotic Ectodermal Dysplasia ◽

Oral Rehabilitation ◽

Treatment Considerations

Abstract Aim This clinical report describes the oral rehabilitation of a 6-year-old male ectodermal dysplasia (ED) patient diagnosed with hypodontia. Background ED is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. Common manifestations include defective hair follicles and eyebrows, frontal bossing with prominent supraorbital ridges, nasal bridge depression, and protuberant lips. Intraorally, most common findings are anadontia or hypodontia, conical teeth, and generalize spaces. The patient may suffer from dry skin, hyperthermia, and unexplained high fever as a result of deficiency of sweat glands. Report A six-year-old boy who exhibited many of the manifestations of ED as well as behavioral problems and a severe gag reflex. The treatment was designed to improve his appearance and oral functions and included the fabrication of several removable prostheses and acid-etched composite resin restorations during his growth and development. Summary Young patients with ED need to be evaluated early by a dental professional to determine the oral ramifications of the condition. When indicated, appropriate care needs to be rendered throughout the child's growth cycle to maintain oral functions as well as to address the esthetic needs of the patient. This clinical report demonstrates that removable partial dentures associated with direct composite restorations can be a reversible and inexpensive method of treatment for young ED patients. Citation Bal C, Bal BT, Casela L, Tüfekçioğlu D. Treatment Considerations for a Patient with Hypohidrotic Ectodermal Dysplasia: A Case Report. J Contemp Dent Pract 2008 March; (9)3:128-134.

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Prosthetic Rehabilitation of a Child Affected from Anhydrotic Ectodermal Dysplasia: A Case Report

The Journal of Contemporary Dental Practice ◽

10.5005/jcdp-6-3-120 ◽

2005 ◽

Vol 6 (3) ◽

pp. 120-126 ◽

Cited By ~ 10

Author(s):

Lorenzo Lo Muzio ◽

Paolo Bucci ◽

Francesco Carile ◽

Francesco Riccitiello ◽

Claudio Scotti ◽

...

Keyword(s):

Case Report ◽

Young Patient ◽

New Technologies ◽

Alveolar Bone ◽

Ectodermal Dysplasia ◽

Composite Resin ◽

Dental Treatment ◽

Clinical Report ◽

Prosthetic Rehabilitation ◽

Dental Tissue

Abstract The aim of this clinical report is to describe the management of a young patient, affected by ectodermal dysplasia, during a nine-year period. Dental treatment can vary depending on the severity of the disease (tooth size, morphology, and amount of available alveolar bone). New technologies, such as adhesive dentistry, and new materials, such as composite resin, represent current options in the management of the dental rehabilitation of patients affected by ectodermal dysplasia. Removable partial dentures were used to replace congenitally missing teeth, and composite resin materials were used to restore conical-shaped maxillary teeth to achieve a favorable esthetic result. This option minimized the sacrifice of healthy dental tissue. Prosthodontic and restorative treatment was provided for the psychological and social comfort of the young patient. Citation Lo Muzio L, Bucci P, Carile F, Riccitiello F, Scotti C, Coccia E, Rappelli G. Prosthetic Rehabilitation of a Child Affected from Anhydrotic Ectodermal Dysplasia: A Case Report. J Contemp Dent Pract 2005 August;(6)3:120-126.

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