Bizarre/Oddities Video: The Snake Babies (Harlequin Type Ichthyosis)

Harlequin-type ichthyosis (also harlequin ichthyosis, ichthyosis congenita, Ichthyosis fetalis or keratosis diffusa fetalis), a , is the most severe form of congenital ichthyosis, characterized by a thickening of the keratin layer in fetal human . In sufferers of the disease, the contains massive, diamond-shaped scales, and tends to have a reddish color. In addition, the eyes, ears, mouth, and other appendages may be abnormally contracted. The scaly keratin greatly limits the child’s movement. Because the is cracked where normal would fold, it is easily pregnable by bacteria and other contaminants, resulting in serious risk of fatal infection.

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The harlequin-type designation comes from both the ’s apparent facial expression and the diamond-shape of the scales (resembling the costume of Arlecchino), which are caused by severe hyperkeratosis. The disease can be diagnosed in the uterus by way of fetal skin biopsy or by morphologic analysis of amniotic fluid cells obtained by amniocentesis. In addition, doctors can now usually recognize common features of the disease through ultrasound, and follow up with 3D ultrasound can diagnose the condition.

http://www.youtube.com/watch?v=8lcCoJmGTCs

http://www.youtube.com/watch?v=47152-qcreQ

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