1/8. Hereditary hypodontia and onychorrhexis of the fingernails and toenail koilonychia: Witkop's tooth-and nail syndrome.The tooth-and-nail syndrome (Witkop's syndrome) is a rare autosomal dominant ectodermal dysplasia manifest by defects of the nail plates of the fingers and toes and hypodontia with normal hair and sweat gland function. We report a thirteen year-old girl who presented with marked longitudinal ridging of the nail plates of all ten fingers. The toenails were mildly ridged with koilonychia. Her mother's fingers were similarly affected to a lesser degree while her toenails appeared normal. Examination of the child's dentition revealed a hyperplastic frenulum and the absence of one of the usual four mandibular incisors. history provided by the mother described the maternal grandmother and maternal great aunt as having identical nail findings and the presence of only three lower incisors. hair examination was normal in the mother and child, and no history or findings of sweat gland dysfunction was present. This report describes familial hypodontia, fingernail onychorrhexis, and toenail koilonychia consistent with Witkop's syndrome.- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |
2/8. Witkop tooth and nail syndrome: report of two cases in a family.tooth and nail syndrome, an autosomal dominant condition, is characterized by hypodontia and nail dysplasia. Mandibular incisors, second molars and maxillary canines are the most frequently missing teeth. tooth shape may vary and conical and narrow crowns are common. Nail dysplasia affects fingernails and toenails and is often more severe in childhood. nails may be spoon-shaped, ridged, slow growing and easily broken. The clinical and radiographic features of a father and son presenting with this rare condition are described.- - - - - - - - - - ranking = 0.8keywords = tooth (Clic here for more details about this article) |
3/8. A rare case of tooth-nail syndrome.The present work is a case report of a 11-year-old patient directed for consultations to Department of Paedodontics, Medical University of Lublin. The clinical examination ascertained numerous lacks of permanent teeth, there were only central incisors in maxilla and first molars in mandibula. With the exception of teeth 54, 55, 64, 74, 84, which were reincluded, the patient had remaining deciduous teeth. The radiographic examination confirmed large oligodontia within permanent teeth. hair, sweat glands, anterior chamber and bottom of the eye were correct. Little disturbances within nail plates of palm fingers were found. A gentle form of ectodermal dysplasia was recognized as a tooth-nail syndrome (Witkop's syndrome).- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |
4/8. Witkop tooth and nail syndrome: a case report.Witkop tooth and nail syndrome is an autosomal dominant ectodermal dysplasia characterized by hypodontia and nail dysplasia. Mutations in MSX-1 have been identified as being involved in the syndrome. Mandibular incisors, secondary molars and maxillary canines are the most frequently missing teeth. tooth shape may vary, and conical and narrow crowns are common. Nail dysplasia affects finger- and toenails, and is often more severe in childhood. nails may be spoon-shaped, rigid, slow-growing and easily broken. The clinical and radiographic features of a mother and child presenting with this rare condition are described.- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |
5/8. Keratin 17 mutation in pachyonychia congenita type 2 patient with early onset steatocystoma multiplex and Hutchinson-like tooth deformity.pachyonychia congenita type 2 (PC-2) is an autosomal dominant disorder characterized by hypertrophic nail dystrophy, focal keratoderma, multiple pilosebaceous cysts, and other features of ectodermal dysplasia. It has been demonstrated that PC-2 is caused by mutations in the keratin 17 and keratin 6b genes. In this report, we describe a missense mutation in the keratin 17 gene, M88T, in a Korean patient whose phenotype included early onset steatocystoma multiplex and Hutchinson-like tooth deformities along with other typical features of PC-2 such as hypertrophic nails, natal teeth and follicular hyperkeratosis.- - - - - - - - - - ranking = 1keywords = tooth (Clic here for more details about this article) |
6/8. Brief clinical report: new, autosomal dominant form of ectodermal dysplasia.We describe a 15-year-old boy with an ectodermal dysplasia syndrome (EDS) associated with unilateral adrenal cyst. The EDS combination of aplasia cutis verticis, hypohidrosis, nipple/breast hypoplasia, onychodysplasia, and delayed dental eruption with minor tooth anomalies has not been previously reported as an autosomal dominant trait. The association with adrenal cyst may alert other units to review their experience to determine if such combination is more than a coincidence.- - - - - - - - - - ranking = 0.2keywords = tooth (Clic here for more details about this article) |
7/8. adult-syndrome: an autosomal-dominant disorder with pigment anomalies, ectrodactyly, nail dysplasia, and hypodontia.We describe a family with at least seven living persons who are affected by an hitherto undescribed autosomal-dominant syndrome with variable expression, bearing close resemblance to the EEC syndrome and related disorders. The main manifestations are hypodontia and/or early loss of permanent teeth, ectrodactyly, obstruction of lacrimal ducts, onychodysplasia, and excessive freckling. We propose the acronym adult (acro-dermato-ungual-lacrimal-tooth)-syndrome for this condition.- - - - - - - - - - ranking = 0.2keywords = tooth (Clic here for more details about this article) |
8/8. Hidrotic ectodermal dysplasia of hair, teeth, and nails: case reports and review.We report two families with the Witkop "tooth and nail syndrome". This term is a misnomer, as the hair was mildly involved in the original case reports and in the families reported here.- - - - - - - - - - ranking = 0.2keywords = tooth (Clic here for more details about this article) |