Multidisciplinary management of hypohydrotic ectodermal dysplasia – a case report

Joseph, Suja; Cherackal, George J.; Jacob, Joby; Varghese, Alex K.

doi:10.1002/ccr3.209

Cited by 17 publications

(36 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…[ 1 ] On the other hand, hidrotic ED is characterized by the triad of onychodysplasia, hypotrichosis, and palmoplantar hyperkeratosis. [ 2 ] The clinical presentation of our patient was consistent with a case of hypohidrotic ED.…”

Section: Discussionsupporting

confidence: 77%

“…However, the two most common subtypes of ED are hypohidrotic/anhidrotic ED in which the sweat glands are deficient and hidrotic ED in which the sweat glands are not affected. [ 1 2 3 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 1 ] The common clinical features of ED include dental abnormalities, hypotrichosis, abnormal nails, and reduced ability to sweat. [ 2 ] As several organ-systems are involved in ED, the treating physician faces several treatment challenges. This case study primarily deals with the management of the disease in the pediatric age group, keeping in track with the latest developments in the field.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

A case report of hypohidrotic ectodermal dysplasia: A mini-review with latest updates

Meshram

Kaur

Hura

2018

J Family Med Prim Care

View full text Add to dashboard Cite

Ectodermal dysplasia (ED) is a rare hereditary disorder involving two or more of the ectodermal structures, which include the skin, hair, nails, teeth, and sweat glands. The two most common forms of the disease are hypohidrotic/anhidrotic ED and hidrotic ED. They are caused by the mutations of several genes. We present a case of a 9-year-old child with hypohidrotic ED, who presented with hypodontia, dyshidrosis, hypotrichosis, and raised body temperature. We treated the raised body temperature symptomatically with cooling techniques and antipyretics. A multidisciplinary approach with physicians from several fields is required to provide comprehensive medical care to patients with ED.

show abstract

Section: Discussionsupporting

confidence: 77%

“…However, the two most common subtypes of ED are hypohidrotic/anhidrotic ED in which the sweat glands are deficient and hidrotic ED in which the sweat glands are not affected. [ 1 2 3 ]…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

A case report of hypohidrotic ectodermal dysplasia: A mini-review with latest updates

Meshram

Kaur

Hura

2018

J Family Med Prim Care

View full text Add to dashboard Cite

show abstract

“…The ability to perspire is reduced due to sweat gland dysfunction, and so patients are predisposed to develop hyperpyrexia due to the misregulation of the body temperature [ 8 , 11 , 16 , 17 ]. This clinical symptom was reported by patients 1 and 2, especially during sports activities or high ambient temperatures.…”

Section: Discussionmentioning

confidence: 99%

Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases

Gomes

Sichi

Giannasi

et al. 2018

Case Reports in Dentistry

View full text Add to dashboard Cite

Ectodermal dysplasia (ED) is a rare hereditary disorder affecting the development of ectoderm-derived organs and tissues. The aim of this study was to describe phenotypic features and the therapeutic approach in dentistry among three patients with ED, correlating their data with the literature. Additionally, to investigate the salivary gland disorders and their impacts on oral microbiota, we performed salivary tests, including salivary flow rate, salivary buffering capacity, and concentration levels of mutans streptococci, lactobacilli, and yeasts. All patients presented oligodontia, resulting in a significant masticatory dysfunction and aesthetic impairment. The counts of mutans streptococci (n=3) and yeasts (n=2) were high; on the other hand, the count of lactobacilli (n=3) was low. Therefore, salivary and microbiological tests showed that the patients with ED, particularly the hypohidrotic type, presented a high risk of enamel caries and susceptibility to oral infections, which may be likely triggered by reduction of salivary flow and/or possible immunological disorders.

show abstract

“…The results are presented in (Table 1). The defects of the case presented can be categorized into Subgroup 1-2 [9].…”

Section: Ectodermal Dysplasia Syndrome With Orthodontic Treatment Andmentioning

confidence: 99%

Ectodermal Dysplasia Syndrome with Orthodontic Treatment and Oral Finds: A Case Report

Li¹,

Li²,

Xu³

2017

JDODT

View full text Add to dashboard Cite

Ectodermal dysplasia, which affects ectodemal derivatives, currently is an X-linked recessive inheritary hypohydrotic ectodermal dysplasia. Patients with ectodermal dysplasia usually characterized by congenital teeth loss, abnormal teeth and inherited through female carriers. In this case report, we present a 5 years old boy of China ethnicity with mandibular anodontia, a rare dental dysplasia in ectodermal dysplasia syndrome. To compromise esthetics and function, a multidisciplinary approach was performed by oral physician, oral pathologist, orthodontist and prosthodontist.

show abstract

Multidisciplinary management of hypohydrotic ectodermal dysplasia – a case report

Cited by 17 publications

References 13 publications

A case report of hypohidrotic ectodermal dysplasia: A mini-review with latest updates

A case report of hypohidrotic ectodermal dysplasia: A mini-review with latest updates

Phenotypic Features and Salivary Parameters in Patients with Ectodermal Dysplasia: Report of Three Cases

Ectodermal Dysplasia Syndrome with Orthodontic Treatment and Oral Finds: A Case Report

Contact Info

Product

Resources

About