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Sklerodermie und Sharp-Syndrom

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Pädiatrische Rheumatologie

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Das Wort ≫scleroderma≪ bedeutet harte Haut. Die Erkrankung Sklerodermie hat jedoch wesentlich mehr Facetten als nur die Verhärtung der Haut. Alle Formen der Sklerodermie sind im Kindesalter selten. Die häufigste Form im Kindesalter ist die lokalisierte Sklerodermie (LS). Sie unterscheidet sich darin von der systemischen Sklerodemie (SSc), dass sie sich meistens nur auf die Haut und das subkutane Gewebe beschränkt. Die SSc ist sehr viel seltener im Kindesalter als die LS, sie zeigt einen progressiven und langfristig tödlichen Verlauf. Eine SSc wird auf Grund der vorläufigen Kriterien des American College of Rheumatology diagnostiziert und in 2 Subtypen aufgeteilt: diffuse systemische Sklerodermie und limitierte systemische Sklerodermie. Das Sharp-Syndrom oder ≫Mixed Connective Tissue Disease≪ (MCTD) stellt eine Mischung verschiedener Komponenten von verschiedenen Kollagenosen dar, wobei häufig eine Komponente der systemischen Sklerodermie vertreten ist.

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Authors and Affiliations

  1. Kinder- und Jugendrheumatische Praxis am Allgemeinen Krankenhaus Eilbeck, Friedrichsberger Str. 60, 22081, Hamburg

    I. Foeldvari (Dr. med.)

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  1. I. Foeldvari
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Editors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, RWTH Aachen, Pauwelsstr. 30, 52074, Aachen

    Norbert Wagner

  2. Olgahospital, Klinikum Stuttgart, Pädiatrisches Zentrum der Landeshauptstadt Stuttgart, Bismarckstr. 8, 70176, Stuttgart

    Günther Dannecker

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Foeldvari, I. (2007). Sklerodermie und Sharp-Syndrom. In: Wagner, N., Dannecker, G. (eds) Pädiatrische Rheumatologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-32815-5_9

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  • DOI: https://doi.org/10.1007/978-3-540-32815-5_9

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