Sweating in ectodermal dysplasia syndromes. A review

Berg, D.

doi:10.1001/archderm.126.8.1075

Cited by 8 publications

(9 citation statements)

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“…[3] Affected males usually have prominent supraorbital ridges, frontal swelling, thin eyebrow, fine-linear wrinkles, sparse hair, detective nails, and saddle nose. [34] Due to the altered anatomy in the lower third of the face, they resemble to edentulous old individuals.…”

Section: Introductionmentioning

confidence: 99%

Oral rehabilitation of a patient with ectodermal dysplasia with prosthodontics treatment

Nallanchakrava

2013

Indian J Dermatol

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Ectodermal dysplasia (ED) is a rare, congenital disease that involves the sweat glands, scalp hair, nails, skin pigmentation, and craniofacial structure. Oral symptoms of ED include multiple tooth abnormalities (such as hypodontia, anadontia, impacted teeth, and peg-shaped or conical anterior teeth) and lack of normal alveolar ridge development. A 12-year-old male patient, in the absence of any other systemic abnormalities, exhibited typical characteristics of ED, visited our department of pedodontics and preventive dentistry at Panineeya Mahavidyalaya Institute of Dental Sciences and Research Centre. In the clinical and radiographic evaluation, it was occurred that he had only maxillary and mandibular first molars in his mouth. A maxillary and mandibular denture with clasps for retention was fabricated for prosthodontic rehabilitation after considering his growth and the number and condition of his present teeth. At the 18-month follow-up no major complications occurred and further future treatment included implants retained fixed partial dentures.

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Section: Introductionmentioning

confidence: 99%

Oral rehabilitation of a patient with ectodermal dysplasia with prosthodontics treatment

Nallanchakrava

2013

Indian J Dermatol

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“…Prosthodontic measures are indicated as early in life as possible, preferably before fi ve years of life; which leads to better self-image, mastication and linguistic development 8 . Mortality rate as high as 30% has been reported in literature during fi rst two years due to respiratory infections and hyperpyrexia 2,9 . Beyond early childhood, the life span of a patient with one of the common types of ectodermal dysplasia is normal to slightly reduced 2,3 .…”

Section: Discussionmentioning

confidence: 99%

A ‘Missed’ Diagnosed Case of Christ-Siemens-Touraine Syndrome

Narayan

2017

J. Nepal Paedtr. Soc.

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Christ-Siemens-Touraine syndrome also known as Anhidrotic ectodermal dysplasia, is a rare genetic disorder, occurring in 1-100,000 live male births. A two year old male child with classical signs and symptoms of the disease, affecting skin, hairs, nails and teeth, misdiagnosed initially as a case of seborrhic dermatitis, and later as tuberculosis, is described here. Treatment includes counselling the family, skin care, avoiding high ambient temperature, and prosthodontic measures. Diagnosis as early as possible, based mainly on clinical features can prevent mortality due to hyperpyrexia and respiratory infections. Beyond early childhood, life expectancy is normal to slightly reduced.

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“…Children with decreased sweating may have a mortality rate of up to 30% in infancy or early childhood because of intermittent hyperpyrexia [6]. No definite pharmacological treatment is available, and the management of affected patients depends on which structures are involved [4].…”

Section: Introductionmentioning

confidence: 99%

An Insight into the Genesis of Hypohidrotic Ectodermal Dysplasia in a Case Report

Kumar¹,

Shetty²,

Dua

et al. 2012

Case Reports in Dentistry

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Hypohidrotic (anhidrotic) ectodermal dysplasia (HED) is a congenital syndrome characterized by sparse hair, oligodontia, and reduced sweating. It is estimated to affect at least one in 17000 people worldwide. We report a rare case of HED in a 14-year-old male child patient which extraorally manifested as alopecia, scanty eyebrow and eye lashes, frontal bossing, depressed nasal bridge, and full and everted lips. Intraoral examination revealed complete anodontia of the deciduous teeth and partial anodontia of the permanent teeth. It is usually inherited as an X-linked recessive trait caused by mutation in any of the three EDA pathway genes. X-linked and autosomal recessive forms are phenotypically similar; thus, identification of carriers of partial forms of the disorder in their families is the key to clarifying intrafamilial genetic transmission.

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Sweating in ectodermal dysplasia syndromes. A review

Cited by 8 publications

References 0 publications

Oral rehabilitation of a patient with ectodermal dysplasia with prosthodontics treatment

Oral rehabilitation of a patient with ectodermal dysplasia with prosthodontics treatment

A ‘Missed’ Diagnosed Case of Christ-Siemens-Touraine Syndrome

An Insight into the Genesis of Hypohidrotic Ectodermal Dysplasia in a Case Report

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