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.

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

NFJS is caused by mutations in the keratin 14 (KRT14) gene, located on chromosome 17q12-21.

[citation needed] In most cases of Naegeli syndrome, a diagnosis is made based on the typical clinical features of this condition.

To avoid overheating, affected individuals should stay hydrated, wear appropriate clothing, and use wet dressings.