1996
DOI: 10.1002/(sici)1096-8628(19960122)61:3<274::aid-ajmg13>3.0.co;2-q
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Clouston syndrome: An ectodermal dysplasia without significant dental findings

Abstract: The ectodermal dysplasias are a heterogeneous group of conditions primarily affecting the hair, teeth, nails, and skin, and are classified according to the tissue(s) affected. The classification categories are: (1) abnormalities of hair, (2) dental defects, (3) abnormalities of nail morphology, and (4) dyshidrosis. Individuals are grouped according to defects present with findings from two or more categories required for diagnosis. As this classification method is based on phenotype, variable expression or sma… Show more

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Cited by 25 publications
(11 citation statements)
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References 6 publications
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“…Most children (n ϭ 102; 72%) had a clinical diagnosis of HED (Christ-Siemens-Touraine syndrome). 11 The remainder had 1 of the following diagnoses: ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome (n ϭ 6), 12 Clouston syndrome (n ϭ 3), 13 Rapp-Hodgkin syndrome (n ϭ 4), 14 oculodentodigital syndrome (n ϭ 3), 15 hidrotic ectodermal dysplasia (n ϭ 1), Hay-Wells syndrome (n ϭ 4), 16 keratitis-ichthyosisdeafness syndrome (n ϭ 1), 17 Gorlin-Goltz syndrome (n ϭ 1), 18 pachyonychia congenita (n ϭ 1), 19 or unclassified (n ϭ 12).…”
Section: Patientsmentioning
confidence: 99%
“…Most children (n ϭ 102; 72%) had a clinical diagnosis of HED (Christ-Siemens-Touraine syndrome). 11 The remainder had 1 of the following diagnoses: ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome (n ϭ 6), 12 Clouston syndrome (n ϭ 3), 13 Rapp-Hodgkin syndrome (n ϭ 4), 14 oculodentodigital syndrome (n ϭ 3), 15 hidrotic ectodermal dysplasia (n ϭ 1), Hay-Wells syndrome (n ϭ 4), 16 keratitis-ichthyosisdeafness syndrome (n ϭ 1), 17 Gorlin-Goltz syndrome (n ϭ 1), 18 pachyonychia congenita (n ϭ 1), 19 or unclassified (n ϭ 12).…”
Section: Patientsmentioning
confidence: 99%
“…Im Gegensatz zu den Xchromosomal vererbten Formen der ektodermalen Dysplasien weisen die meisten Patienten eine normale Zahnentwicklung und eine normale Funktion der Schweiß-und Talgdrüsen auf.Hingegen finden sich pathologische Veränderungen der Haare bis hin zur Alopecia totalis, eine schwere Nageldystrophie und eine Hyperpigmentierung der Haut über den großen Gelenken (⊡ Abb.6).Mitunter werden auch palmare Hyperkeratosen, Strabismus und mentale Retardierung beobachtet [56].…”
Section: Clouston-syndromunclassified
“…Usually the patients present with thickened, ridged, discolored, hyperconvex, brittle, or small nails. Nail deformities are frequently associated with paronychia …”
Section: Introductionmentioning
confidence: 99%