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Porphyrien

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Therapie der Hautkrankheiten

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Zusammenfassung

Die Porphyrien stellen eine nicht allzu seltene Gruppe von Stoffwechselkrankheiten dar, in denen stark erhöhte Mengen von Porphyrinen oder ihrer Vorstufen im Körper gebildet werden. Ursache dafür sind Störungen der Hämbiosynthese, die auf genetisch bedingten, spezifischen Enzymdefekten beruhen. Teilweise werden diese genetischen Defekte erst dann manifest, wenn eine zusätzliche Belastung durch Arzneimittel oder andere Umweltschadstoffe hinzukommt. Da die Porphyrine phototoxische Substanzen sind, die zum Teil in der Haut in erhöhtem Maße vorhanden sind bzw dort gelagert werden, ist das klinische Leitsymptom der meisten Porphyrien eine phototoxische Hautreaktion.

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  1. Universitäts-Hautklinik und Poliklinik, Klinikum Benjamin Franklin der Freien Universität Berlin, Hindenburgdamm 30, 12200, Berlin, Deutschland

    Prof. Dr. Prof. h.c. Constantin E. Orfanos & Prof. Dr. Claus Garbe

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Orfanos, C.E., Garbe, C. (1995). Porphyrien. In: Therapie der Hautkrankheiten. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-97602-5_34

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  • DOI: https://doi.org/10.1007/978-3-642-97602-5_34

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