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Harlequin's syndrome

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An autosomal recessive form of ichtyosis that is incompatible with extrauterine life. The child is born with abnormal skin which is divided into areas by deep red fissures, diamond-shaped plaques, up to 4-5 cm on one side, that resembles a thick horny armor. The affected infants are usually of low birth-weight and die within 1 week after birth. Aetiology unknown. Inheritance is autosomal recessive.


  • E. Seeligmann:
    De epidermis imprimis neonatorum desquamatione.
    Inaugural dissertation. Berlin, 1841.
    [Epidermal desquamation of the newborn.]
  • E. Riecke:
    Über Ichtyosis congenita.
    Archiv für Dermatologie und Syphilis, Berlin, 1900, 54: 289-340.
  • M. S. Thomson, C. P. G. Wakely:
    The harlequin fetus. The Journal of Obstetrics and Gynecology of the British Empire, London, 1921, 28: 190-203.

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