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Sklerodermie bei Kindern und Jugendlichen

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Pädiatrische Rheumatologie

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Zusammenfassung

Das Wort „scleroderma“ bedeutet harte Haut. Die Erkrankung Sklerodermie hat jedoch wesentlich mehr Facetten als nur eine Verhärtung der Haut. Alle Formen der Sklerodermie sind im Kindesalter selten. Die häufigste Form im Kindesalter ist die lokalisierte Sklerodermie (LS). Sie unterscheidet sich von der systemischen Sklerodermie (SSc) dadurch, dass sie sich meistens auf die Haut und das subkutane Gewebe beschränkt; ungefähr 30 % der Patienten haben eine extrakutane Beteiligung. Die SSc ist sehr viel seltener im Kindesalter als die LS, sie zeigt einen progressiven und langfristig möglicherweise tödlichen Verlauf. Eine SSc wird aufgrund der Kriterien des American College of Rheumatology und/oder der Klassifikationskriterien für juvenile systemische Sklerodermie diagnostiziert und in 2 Subtypen nach Leroy aufgeteilt: diffuse systemische Sklerodermie und limitierte systemische Sklerodermie. Es sind aktuelle Empfehlungen bezüglich Diagnose, Verlaufskontrollen und Therapie erschienen.

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  1. Hamburger Zentrum für Kinder- und Jugendrheumatologie, Schön Klinik Eilbek, Hamburg, Deutschland

    Ivan Foeldvari

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  1. Ivan Foeldvari
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  1. Universitätsklinikum Aachen Klinik für Kinder- und Jugendmedizin, Aachen, Germany

    Norbert Wagner

  2. 15306 Seelow, Germany

    Günther Dannecker

  3. Kinderklinik, Charité Universitätsmedizin Berlin, Berlin, Berlin, Germany

    Tilmann Kallinich

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Foeldvari, I. (2021). Sklerodermie bei Kindern und Jugendlichen. In: Wagner, N., Dannecker, G., Kallinich, T. (eds) Pädiatrische Rheumatologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-60411-3_41-1

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  • DOI: https://doi.org/10.1007/978-3-662-60411-3_41-1

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  • Print ISBN: 978-3-662-60411-3

  • Online ISBN: 978-3-662-60411-3

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