scholarly journals EDA mutation as a cause of hypohidrotic ectodermal dysplasia: a case report and review of the literature

2015 ◽  
Vol 14 (3) ◽  
pp. 10344-10351 ◽  
Author(s):  
S.X. Huang ◽  
J.L. Liang ◽  
W.G. Sui ◽  
H. Lin ◽  
W. Xue ◽  
...  
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Review Of The Literature ◽  
Hypohidrotic Ectodermal Dysplasia

scholarly journals Ectodermal dysplasia - Maxillary and mandibular alveolar reconstruction with dental rehabilitation: A case report and review of the literature

2010 ◽  
Vol 43 (01) ◽  
pp. 092-096
Author(s):  
Sanjeev N. Deshpande ◽  
Vikas Kumar
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Review Of The Literature ◽  
Hypohidrotic Ectodermal Dysplasia ◽  
Inherited Disorders ◽  
Oral Rehabilitation ◽  
Osseointegrated Implants ◽  
Dental Rehabilitation ◽  
Free Fibula ◽  
Rare Group

ABSTRACTEctodermal dysplasia is a rare group of inherited disorders characterized by aplasia or dysplasia of tissues of ectodermal origin, such as hair, nails, teeth and skin. Dental manifestations include hypodontia, complete anodontia or malformed teeth. Oral rehabilitation is the major surgical challenge in such patients. It frequently requires alveolar reconstruction followed by dental implants. We report a case of hypohidrotic ectodermal dysplasia, which was managed with reconstruction of both the upper and the lower alveolus using free fibula flaps with dental rehabilitation using osseointegrated implants.

Clinical management of hypohidrotic ectodermal dysplasia with anodontia: case report

2003 ◽  
Vol 27 (1) ◽  
pp. 5-8 ◽  
Author(s):  
Ekaterini Paschos ◽  
Karin Christine Huth ◽  
Reinhard Hickel
Keyword(s):  
Case Report ◽  
Young Child ◽  
Clinical Management ◽  
Ectodermal Dysplasia ◽  
Psychological Development ◽  
Early Age ◽  
Complete Dentures ◽  
Hypohidrotic Ectodermal Dysplasia ◽  
Need For Treatment ◽  
Stomatognathic System

Ectodermal dysplasia is a rare hereditary disorder with a characteristic physiognomy. The case of a 5-year-old child with hypohidrotic ectodermal dysplasia and complete anodontia is presented. Because of the anodontia and the need for treatment at an early age, the prosthetic management of such a young child can be difficult. Complete dentures were provided to encourage a normal psychological development and to improve the function of the stomatognathic system.

scholarly journals Christ-Siemens-Touraine Syndrome: A Case Report and Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-3 ◽  
Author(s):  
Sepideh Mokhtari ◽  
Saeedeh Mokhtari ◽  
Ali Lotfi
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Classical Case ◽  
Common Type ◽  
Rare Disorder ◽  
Review Of The Literature ◽  
Sebaceous Glands ◽  
Dental Abnormalities ◽  
Anhidrotic Ectodermal Dysplasia

Ectodermal dysplasia is a rare disorder with defects in two or more of the following structures: the teeth and the skin and its appendages including hair, nails, eccrine, and sebaceous glands. Anhidrotic ectodermal dysplasia is the most common type of disease. This rare disorder, also known as Christ-Siemens-Touraine syndrome, manifests as a triad of hypotrichosis, asteatosis, and anhidrosis. In view of the rarity of this entity, a classical case of anhidrotic ectodermal dysplasia is reported. We have also provided a review of recent investigations in the area of dental abnormalities in this syndrome.

Case report: Higher-than-expected prevalence of hypohidrotic ectodermal dysplasia in a rural Mexican setting: report of 20 cases

2010 ◽  
Vol 49 (9) ◽  
pp. 1050-1055 ◽  
Author(s):  
Nayellin Reyes-Chicuellar ◽  
Roberto De Haro ◽  
Rafael Jiménez-Flores ◽  
Jovita Monje-Martínez ◽  
Norma Motta-Castañeda ◽  
...  
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Hypohidrotic Ectodermal Dysplasia

scholarly journals A novel EDA variant causing X-linked hypohidrotic ectodermal dysplasia: Case report

2021 ◽  
Vol 29 ◽  
pp. 100796
Author(s):  
Baiba Alksere ◽  
Liene Kornejeva ◽  
Ieva Grinfelde ◽  
Aigars Dzalbs ◽  
Dace Enkure ◽  
...  
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Hypohidrotic Ectodermal Dysplasia

scholarly journals Ectodermal Dysplasia presenting with Atrophic Rhinitis: A Report of Two Cases and a Review of the Literature

2016 ◽  
Vol 9 (1) ◽  
pp. 53-55
Author(s):  
Sampurna Pati ◽  
Sudipta Pal ◽  
Surajit Biswas ◽  
Somnath Saha
Keyword(s):  
Case Report ◽  
Conservative Therapy ◽  
Ectodermal Dysplasia ◽  
Tertiary Care ◽  
Atrophic Rhinitis ◽  
Mucosal Biopsy ◽  
Review Of The Literature ◽  
History Of ◽  
Conventional Manner ◽  
Nose And Throat

ABSTRACT Aims To present two rare cases of ectodermal dysplasia presenting with maggot infestation due to atrophic rhinitis. Study design Case report. Setting A tertiary care referral hospital. Presentation Two male children aged about 5 and 8 years presented to the ear, nose, and throat emergency with a history of maggot infestation of the nose. Results Clinical examination was suggestive of ectodermal dysplasia. Intranasal examination was suggestive of atrophic rhinitis. Maggots were removed in the conventional manner. Following a conservative treatment, skin biopsy and nasal mucosal biopsy were done, which confirmed the diagnosis of ectodermal dysplasia. Conclusion Any ectodermal dysplasia patient should be suspected of having atrophic rhinitis and intranasal conservative therapy should be initiated at the earliest to prevent complications like maggots in the nose. How to cite this article Pati S, Pal S, Saha S, Biswas S. Ectodermal Dysplasia presenting with Atrophic Rhinitis: A Report of Two Cases and a Review of the Literature. Clin Rhinol An Int J 2016;9(1):53-55.

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

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.

scholarly journals Prosthodontic management of hypohidrotic ectodermal dysplasia: a case report

2017 ◽  
Vol 2 (2) ◽  
Author(s):  
Emilija Bajraktarova Valjakova ◽  
Cvetanka Misevska ◽  
Vesna Korunoska Stevkovska ◽  
Nikola Gigovski ◽  
Ana Sotirovska Ivkovska ◽  
...  
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Hypohidrotic Ectodermal Dysplasia

Ectrodactyly-ectodermal dysplasia-clefting syndrome

2000 ◽  
Vol 90 (9) ◽  
pp. 460-464 ◽  
Author(s):  
GJ Kelman ◽  
RC Aronoff
Keyword(s):  
Congenital Anomaly ◽  
Case Report ◽  
Female Patient ◽  
Surgical Management ◽  
Ectodermal Dysplasia ◽  
Review Of The Literature ◽  
Rare Congenital Anomaly ◽  
Rare Syndrome ◽  
Clinical Triad ◽  
Musculoskeletal Involvement

Ectrodactyly-ectodermal dysplasia-clefting syndrome is a rare congenital anomaly that affects tissues of mesodermal and ectodermal origin. Musculoskeletal involvement frequently requires orthopedic intervention. The authors present a review of the literature pertaining to this rare syndrome as well as a case report of a female patient who exhibited the complete clinical triad. A description of the surgical management of her condition is also presented.

scholarly journals Oral rehabilitation with removable dental prosthesis in a six-year-old patient with hypohidrotic ectodermal dysplasia

RSBO ◽  
2017 ◽  
Vol 14 (1) ◽  
pp. 44-09
Author(s):  
Mariana Dalledone ◽  
Fernanda Mara de Paiva Bertoli ◽  
Ana Paula Sponchiado ◽  
Estela Maris Losso ◽  
João Armando Brancher ◽  
...  
Keyword(s):  
Case Report ◽  
Ectodermal Dysplasia ◽  
Self Esteem ◽  
Vertical Dimension ◽  
Hypohidrotic Ectodermal Dysplasia ◽  
Oral Rehabilitation ◽  
Palatal Expansion ◽  
Expansion Procedure ◽  
Removable Dental Prosthesis

Ectodermal dysplasia is an abnormality of the ectoderm and its derivatives, phenotypically expressed in males and genetically inherited from mothers. Objective: To report the efficacy of the prosthetics treatment of severe ectodermal dysplasia in a 6-year-old boy with hypohidrotic ectodermal dysplasia (HED). Case report: The boy had difficulty in speaking and eating and the condition also affected his self-esteem. Prosthetic treatments included the construction of acrylic custom trays, determination of the vertical dimension of occlusion (VDO), construction of upper and lower dentures, and palatal expansion procedure. Conclusion: Significant improvements in the patient’s stomatognathic function and self-esteem were evident.

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