ektodermální dysplazie [Ectodermal Dysplasia]

tematický
33
Termíny

anhidrotická ektodermální dysplazie
aplasia cutis congenita
Cloustonův syndrom
hidrotická ektodermální dysplazie
vrozená ektodermální dysplazie

 

Anhidrotic Ectodermal Dysplasia
Anhydrotic Ectodermal Dysplasia
Aplasia Cutis Congenita
Aplasia Cutis Congenita, Nonsyndromic
Autosomal Dominant Hidrotic Ectodermal Dysplasia
Clouston Hidrotic Ectodermal Dysplasia
Clouston Syndrome
Clouston's Hidrotic Ectodermal Dysplasia
Clouston's Syndrome
Congenital Ectodermal Defect
Defect, Congenital Ectodermal
Ectodermal Defect, Congenital
Ectodermal Dysplasia 2, Hidrotic
Ectodermal Dysplasia Anhidrotic
Ectodermal Dysplasia, Anhydrotic
Ectodermal Dysplasia, Hidrotic
Ectodermal Dysplasia, Hidrotic, Autosomal Dominant
Hidrotic Ectodermal Dysplasia
Hidrotic Ectodermal Dysplasia, Autosomal Dominant
Hydrotic Ectodermal Dysplasia

Perzistentní odkaz   https://www.medvik.cz/link/D004476
Definice

Vývojová porucha ektodermu s různým postižením tkání z něho odvozených. V současnosti se popisuje přes 100 různých syndromů podle šíře a kombinace postižení. V klasifikaci e. d. se někdy používá číselné kombinace postižení jednotlivých složek: vlasy (1), zuby (2), nehty (3) a potní žlázy (4), k nimž jsou jednotlivé syndromy přiřazovány. (cit. Velký lékařský slovník online, 2017 http://lekarske.slovniky.cz)

A group of hereditary disorders involving tissues and structures derived from the embryonic ectoderm. They are characterized by the presence of abnormalities at birth and involvement of both the epidermis and skin appendages. They are generally nonprogressive and diffuse. Various forms exist, including anhidrotic and hidrotic dysplasias, FOCAL DERMAL HYPOPLASIA, and aplasia cutis congenita.

DUI
D004476 MeSH Prohlížeč
CUI
M0007032
Historická pozn.
1965
Veřejná pozn.
1965; ECTODERMAL DEFECT, CONGENITAL was heading 1963-1986

C Nemoci
C16.131 vrozené vady 1 740
C16.131.077.019 syndrom delece 22q11 2
C16.131.077.065 Alagillův syndrom 19
C16.131.077.095 Angelmanův syndrom 35
C16.131.077.121 Barthův syndrom 6
C16.131.077.137 Bloomův syndrom 11
C16.131.077.229 Carneyův komplex 8
C16.131.077.245 ciliopatie 9
C16.131.077.250 Cockayneův syndrom 4
C16.131.077.256 Costellův syndrom 3
C16.131.077.272 de Langeové syndrom 4
C16.131.077.299 hluchoslepota 65
C16.131.077.313 Donohueův syndrom 1
C16.131.077.327 Downův syndrom 510
C16.131.077.350 ektodermální dysplazie 33
C16.131.077.350.398 Ellisův-van Creveldův syndrom 11
C16.131.077.350.424 fokální dermální hypoplazie 6
C16.131.077.350.712 neurokutánní syndromy 29
C16.131.077.350.856 pachyonychia congenita 3
C16.131.077.371 Fraserův syndrom 2
C16.131.077.393 Gardnerův syndrom 20
C16.131.077.401 heterotaxe 7
C16.131.077.410 holoprosencefalie 8
C16.131.077.445 incontinentia pigmenti 19
C16.131.077.537 Loeysův-Dietzův syndrom 7
C16.131.077.550 Marfanův syndrom 105
C16.131.077.578 Moebiův syndrom 10
C16.131.077.592 moniletrichosis 1
C16.131.077.619 Nethertonův syndrom 2
C16.131.077.696 Cantrellova pentalogie 1
C16.131.077.703 POEMS syndrom 34
C16.131.077.730 Praderův-Williho syndrom 91
C16.131.077.735 nedostatek prolidázy
C16.131.077.740 Proteův syndrom 8
C16.131.077.745 prune belly syndrom 3
C16.131.077.790 zarděnky vrozené 20
C16.131.077.889 Sotosův syndrom 4
C16.131.077.919 Patauův syndrom 6
C16.131.077.929 syndrom trizomie 18 5
C16.131.077.938 Waardenburgův syndrom 10
C16.131.077.970 Zellwegerův syndrom 10
C16.131.831 kožní abnormality 51
C16.131.831.066 akrodermatitida 66
C16.131.831.108 Carneyův komplex 8
C16.131.831.150 dyskeratosis congenita 9
C16.131.831.350 ektodermální dysplazie 33
C16.131.831.350.398 Ellisův-van Creveldův syndrom 11
C16.131.831.350.424 fokální dermální hypoplazie 6
C16.131.831.350.712 neurokutánní syndromy 29
C16.131.831.350.856 pachyonychia congenita 3
C16.131.831.493 epidermolysis bullosa 108
C16.131.831.512 ichtyóza 62
C16.131.831.580 incontinentia pigmenti 19
C16.131.831.675 nevus flammeus 5
C16.131.831.720 nedostatek prolidázy
C16.131.831.766 pseudoxanthoma elasticum 13
C16.131.831.812 sclerema neonatorum 2
C16.131.831.936 xeroderma pigmentosum 25
C16.320.850.080 albinismus 17
C16.320.850.180 cutis laxa 10
C16.320.850.190 Darierova nemoc 26
C16.320.850.210 atopická dermatitida 1 444
C16.320.850.235 dyskeratosis congenita 9
C16.320.850.250 ektodermální dysplazie 33
C16.320.850.250.398 Ellisův-van Creveldův syndrom 11
C16.320.850.250.424 fokální dermální hypoplazie 6
C16.320.850.250.712 neurokutánní syndromy 29
C16.320.850.250.856 pachyonychia congenita 3
C16.320.850.275 epidermolysis bullosa 108
C16.320.850.368 systémová hyalinóza 1
C16.320.850.405 ichthyosis vulgaris 5
C16.320.850.420 incontinentia pigmenti 19
C16.320.850.595 Urbachova-Wietheova nemoc 5
C16.320.850.647 moniletrichosis 1
C16.320.850.673 Nethertonův syndrom 2
C16.320.850.730 porokeratóza 11
C16.320.850.738 erytropoetická porfyrie 11
C16.320.850.742 jaterní porfyrie 26
C16.320.850.746 nedostatek prolidázy
C16.320.850.750 pseudoxanthoma elasticum 13
C16.320.850.970 xeroderma pigmentosum 25
C17.800 kožní nemoci 2 796
C17.800.804 kožní abnormality 51
C17.800.804.066 akrodermatitida 66
C17.800.804.108 anetodermie 5
C17.800.804.150 dyskeratosis congenita 9
C17.800.804.350 ektodermální dysplazie 33
C17.800.804.350.398 Ellisův-van Creveldův syndrom 11
C17.800.804.350.424 fokální dermální hypoplazie 6
C17.800.804.350.712 neurokutánní syndromy 29
C17.800.804.350.856 pachyonychia congenita 3
C17.800.804.493 epidermolysis bullosa 108
C17.800.804.512 ichtyóza 62
C17.800.804.580 incontinentia pigmenti 19
C17.800.804.675 nevus flammeus 5
C17.800.804.766 pseudoxanthoma elasticum 13
C17.800.804.812 sclerema neonatorum 2
C17.800.804.936 xeroderma pigmentosum 25
C17.800.827.080 albinismus 17
C17.800.827.180 cutis laxa 10
C17.800.827.190 Darierova nemoc 26
C17.800.827.210 atopická dermatitida 1 444
C17.800.827.235 dyskeratosis congenita 9
C17.800.827.250 ektodermální dysplazie 33
C17.800.827.250.398 Ellisův-van Creveldův syndrom 11
C17.800.827.250.424 fokální dermální hypoplazie 6
C17.800.827.250.712 neurokutánní syndromy 29
C17.800.827.250.856 pachyonychia congenita 3
C17.800.827.275 epidermolysis bullosa 108
C17.800.827.384 systémová hyalinóza 1
C17.800.827.405 ichthyosis vulgaris 5
C17.800.827.420 incontinentia pigmenti 19
C17.800.827.602 moniletrichosis 1
C17.800.827.610 Torrého-Muirův syndrom 10
C17.800.827.655 Nethertonův syndrom 2
C17.800.827.730 porokeratóza 11
C17.800.827.738 erytropoetická porfyrie 11
C17.800.827.742 jaterní porfyrie 26
C17.800.827.750 pseudoxanthoma elasticum 13
C17.800.827.970 xeroderma pigmentosum 25