incontinentia pigmenti [Incontinentia Pigmenti]

tematický
19
Termíny

Blochův-Sulzbergerův syndrom
melanoblastosis Bloch-Sulzberg
melanoblastosis cutis linearis sive systematica
naevus pigmentosus systematicus
Siemensův-Blochův-Sulzbergův syndrom

 

Bloch-Siemens Syndrome
Bloch-Siemens-Sulzberger Syndrome
Bloch-Sulzberger Syndrome

Perzistentní odkaz   https://www.medvik.cz/link/D007184
Definice

Familiárně podmíněné onemocnění (podle některých pramenů gonozomálně dědičné a letální u mužů, jiné prameny uvádějí dědičnost autozomálně dominantní) patrné obv. již po narození. Probíhá v několika etapách bulózní stadium, hyperkeratotické změny s papulami a bradavicemi, změny pigmentace (vytvářejí bizarní ložiska). Nápadná bývá eozinofilie v krvi. Mohou být přítomny i další defekty (zubů, očí, CNS, kostry). (cit. Velký lékařský slovník online, 2017 http://lekarske.slovniky.cz)

A genodermatosis occurring mostly in females and characterized by skin changes in three phases - vesiculobullous, verrucous papillomatous, and macular melanodermic. Hyperpigmentation is bizarre and irregular. Sixty percent of patients have abnormalities of eyes, teeth, central nervous system, and skin appendages.

Anotace
multiple skin & other abnorm; do not confuse with INCONTINENTIA PIGMENTI ACHROMIANS see PIGMENTATION DISORDERS
DUI
D007184 MeSH Prohlížeč
CUI
M0011200
Předchozí užití
Pigmentation Disorders (1966-1986)
Historická pozn.
91(87); was see under PIGMENTATION DISORDERS 1987-90
Veřejná pozn.
91; was see under PIGMENTATION DISORDERS 1987-90

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
C16.131.077.371 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
C16.131.831.493 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
C16.320.850.275 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
C17.800.621 poruchy pigmentace 132
C17.800.621.166 argyrie 13
C17.800.621.250 skvrny bílé kávy 14
C17.800.621.430 hyperpigmentace 65
C17.800.621.440 hypopigmentace 14
C17.800.621.497 incontinentia pigmenti 19
C17.800.621.893 urticaria pigmentosa 18
C17.800.621.936 xeroderma pigmentosum 25
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
C17.800.804.493 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
C17.800.827.275 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