Witkop tooth and nail syndrome: report of two cases in a family

Hodges, SJ; Harley, Kathryn

doi:10.1046/j.1365-263x.1999.00128.x

Cited by 16 publications

(16 citation statements)

References 6 publications

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“…Tooth shape has been described as parallel‐sided or tapering toward the incisal edge. These morphological changes have been noted in both the primary and permanent dentitions (5). Tapering teeth were seen in all the individuals presented here, although different permanent teeth were affected.…”

Section: Discussionmentioning

confidence: 95%

“…Genetic counseling is beneficial for affected individuals and their families (3). Other treatments for this disorder are symptomatic and supportive in nature (5,8,14). Treatment may primarily consist of dental restoration.…”

Section: Discussionmentioning

confidence: 99%

“…However, in contrast to people with EDs, people with Witkop syndrome have normal hair and normal sweat gland function (4). A further difference compared with severe forms of ED is that no typical facial appearance is associated with Witkop syndrome (5). Typical characteristics of the latter include nail dysplasia and hypodontia.…”

mentioning

confidence: 99%

“…Isolated cases of Witkop syndrome without a positive family history have been reported (5). We describe here the clinical and radiographic features of three siblings – a boy and two girls – with this rare condition who were seen at the Department of Pediatric Dentistry, Shiraz Dental School, Shiraz University of Medical Sciences, in Shiraz, Iran.…”

mentioning

confidence: 99%

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Witkop Tooth and Nail Syndrome: A Report of Three Cases in a Family

Memarpour

Shafıei

2010

Pediatric Dermatology

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Witkop tooth and nail syndrome is a rare autosomal dominant ectodermal dysplasia manifested by hypodontia and nail dysplasia. Tooth shape may vary, and conical and narrow crowns are common. Mandibular incisors, second molars, and maxillary canines are the most frequently missing teeth. Nail dysplasia affects the fingernails and especially the toenails and is often more severe in childhood. Nails may be spoon-shaped, ridged, slow-growing, and easily broken. We examined an Eastern Mediterranean family in which three siblings (two girls, one boy) had this syndrome. The severity of finger and toenail anomalies varied. The children's mother had no Witkop-related abnormalities, but their father was reported to have dental and nail anomalies. Early intervention is recommended to manage orofacial disfigurement and avoid negative social consequences for these children.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Witkop Tooth and Nail Syndrome: A Report of Three Cases in a Family

Memarpour

Shafıei

2010

Pediatric Dermatology

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“…The Witkop syndrome is a rare autosomal dominant ED characterized by hypodontia and nail dysplasia [Hodges and Harley, 1999]. The number of missing teeth and types of anomalies identified is extremely variable, but premolar, first molars and third molars are most often involved.…”

Section: Transcription Factorsmentioning

confidence: 99%

Ectodermal dysplasias: An overview and update of clinical and molecular‐functional mechanisms

Priolo

2009

American J of Med Genetics Pt A

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The ectodermal dysplasias (EDs) are a large and complex group of disorders. In various combinations, they all share anomalies in hair, teeth, nails, and sweat gland function. The anomalies affecting the epidermis and epidermal appendages are extremely variable. Many are associated with malformations in other organs and systems. Clinical overlap is present among EDs. Few causative genes have been identified, to date. Most of the EDs present multisystem involvement with abnormal development of structures also derived from mesoderm. In the last few years, it has become evident that gene expression in the EDs is not limited to the ectoderm and that there is a concomitant effect on developing mesenchymal structures, with modification or abolition of ectodermal-mesenchymal signaling. It is possible to approach this group of diseases basing on functional and molecular findings and to begin to explain the complex clinical consequences of mutations affecting specific developmental pathways. We have reviewed the molecular basis of ectodermal dysplasias applying this new clinical-functional classification. For each subset of the identified ED, we will now describe the genes and related proteins involved in terms of: (1) structure of the genes and their role in differentiation of the epidermis and the ectodermal derivatives; (2) genotype-phenotype correlation.

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