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Naegeli's syndrome

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Syndrome characterized by reticular skin changes, dental anomalies, disturbance of temperature regulation due to diminished function of the sweat glands, hyperkeratosis palmaris and plantaris, nystagmus, strabismus, and optic atrophy. Onset in 2nd or 3rd year of life, usually involving first the trunk and upper extremities, from where it spreads to other parts. Both sexes affected with equal frequency. Inheritance is autosomal dominant.

Naegeli described the condition in 1927 as "melanophoric nevus".


  • O. Naegeli:
    Familiärer Chromatophorennaevus.
    Schweizerische medizinische Wochenschrift, Basel, 1927, 57: 48.
  • A. Franceschetti, W. Jadassohn:
    A propos de «l’incontinentia pigmenti,» délimitation de deux syndromes différents figurant sous le même terme.
    Dermatologica, Basel, 1954, 108: 1-28.

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