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Naegeli–Franceschetti–Jadassohn syndrome (also known as "Chromatophore nevus of Naegeli"^[1]:548)^[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.

Naegeli syndrome is similar to Dermatopathia pigmentosa reticularis,^[4] both of which are caused by a specific defect in the keratin 14 protein.

Naegeli–Franceschetti–Jadassohn syndrome has an autosomal dominant pattern of inheritance.

[edit] Eponym

It was named after Oskar Naegeli.^[5]

[edit] See also

• Josef Jadassohn

[edit] References

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