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Sclérose tubéreuse de Bourneville

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Manifestations dermatologiques des maladies d’organes

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Résumé

La sclérose tubéreuse de Bourneville (STB) est une affection caractérisée par la survenue de tumeurs bénignes (hamartomes) dans divers tissus, secondaire à la mutation de gènes suppresseurs de tumeurs TSC1 et TSC2. Les organes préférentiellement atteints sont la peau, le cerveau, les reins, les yeux et le coeur. La première description est attribuée à Bourneville en1880, qui donna son nom à cette maladie en se référant aux lésions tubéreuses (tubers corticaux) au niveau cérébral. Mais c’est en 1908 que Vogt présenta la classique triade: épilepsie, retardmental, adénomes sébacés.

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Authors
  1. Fabienne Ballanger
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  2. Gaëlle Quereux
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  3. Sébastien Barbarot
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© 2012 Springer-Verlag France, Paris

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Ballanger, F., Quereux, G., Barbarot, S. (2012). Sclérose tubéreuse de Bourneville. In: Manifestations dermatologiques des maladies d’organes. Springer, Paris. https://doi.org/10.1007/978-2-287-72073-4_22

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  • DOI: https://doi.org/10.1007/978-2-287-72073-4_22

  • Publisher Name: Springer, Paris

  • Print ISBN: 978-2-287-72072-7

  • Online ISBN: 978-2-287-72073-4

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