Naegeli–Franceschetti–Jadassohn syndrome
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Naegeli–Franceschetti–Jadassohn syndrome (NFJS), also known as chromatophore nevus of Naegeli and Naegeli syndrome,[1][2] is a rare autosomal dominant[3] form of ectodermal dysplasia, characterized by reticular skin pigmentation, diminished function of the sweat glands, the absence of teeth and hyperkeratosis of the palms and soles. One of the most striking features is the absence of fingerprint lines on the fingers.
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Naegeli–Franceschetti–Jadassohn syndrome | |
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Other names | Chromatophore nevus of Naegeli |
Naegeli–Franceschetti–Jadassohn syndrome has an autosomal dominant pattern of inheritance |
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Naegeli syndrome is similar to dermatopathia pigmentosa reticularis,[4] both of which are caused by a specific defect in the keratin 14 protein.