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Touraine's syndrome I

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Neurocutaneous phacomatosis with spread of naevus cells in the skin and diffuse or circumscribed pigmentation of the leptomeninges by naevus cells. Frequently, scondary hydrocephalus internus.

First described in 1861 by Rudolf Virchow (1821-1902) and 1861 by Karl von Rokitansky (1804-1878). The term “neurocutaneous melanoblastoma” was coined by Albert Touraine in 1941 and thus the disease was added to the neurocutaneous syndromes.


  • R. Virchow:
    Pigment und diffuse Mélanose der Arachnoides.
    [Virchows] Archiv für pathologische Anatomie und Physiologie und für klinische Medicin, Berlin, 1859, 16: 180-187.
  • K. von Rokitansky:
    Ein ausgezeichneter Fall von Pigment-Mal mit ausgebreiteter Pigmentierung der inneren Hirn- und Rückenmarkshäute.
    Allgemeine Wiener medizinische Zeitung, 1861, 15: 113-116.
  • A. Touraine:
    La Mélanoblastose neurocutanée.
    Presse médicale, Paris, 1941, 49: 1087-1088.

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