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Das Basalzellennaevus-Syndrom (BCNS)

  • Chapter
Nicht Entzündliche Dermatosen III/A

Part of the book series: Handbuch der Haut- und Geschlechtskrankheiten ((1656,volume 3 / 3 / a))

Zusammenfassung

Beim sogenannten Basalzellennaevus-Syndrom (BCNS) handelt es sich um ein polysymptomatisches, autosomal-dominant vererbtes Krankheitsbild, das in wechselnder Ausprägung Haut, Auge, ZNS, Skelettsystem, Bindegewebe und endokrine Organe befällt, daneben aber noch eine Reihe uncharakteristischer Symptome aufweisen kann. Dies haben umfangreiche Untersuchungen befallener Familien ergeben (Gorlin et al., 1965; Maddox, 1962; Anderson, McClendon u. Howell 1964; Gerber, 1965; Berlin, van Scott et al., 1966). Hermans et al. (1959, 1960, 1963, 1965 a, b) haben eine Einordnung unter die Phakomatosen verlangt; Herzberg u. Wiskemann haben sich dieser Forderung angeschlossen.

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Holubar, K. (1975). Das Basalzellennaevus-Syndrom (BCNS). In: Holubar, K., et al. Nicht Entzündliche Dermatosen III/A. Handbuch der Haut- und Geschlechtskrankheiten, vol 3 / 3 / a. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-30477-8_4

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