Naegeli-Franceschetti-Jadassohn syndrome

background

Naegeli-Franceschetti-Jadassohn (NFJ) syndrome is a form of ectodermal dysplasia that affects the outer layer of a developing baby called the ectoderm. Because the ectodermal layer develops into many parts of the baby's body, including the lens of the eye, parts of the inner ear, fingers, toes, and nerves, these parts may not develop normally.
NFJ syndrome is very rare. It affects only one in 2-4 million people and has been found among families in Switzerland, Japan, Italy, and Greece. Males and females are equally affected.
NFJ syndrome affects the skin, sweat glands, nails, and teeth. Other problems include lack of fingerprints, thickened skin on the palms of the hands and soles of the feet, and fingernail and dental problems.
NFJ was named after the first few scientists who studied it. The symptoms of NFJ syndrome were first described in a Swiss family by Naegeli in 1927. Franceschetti and Jadassohn added to the knowledge of NFJ in the 1950s.
People with NFJ syndrome are of normal intelligence and tend to be in otherwise good health. Although there is no cure for NFJ, most people with the disorder can expect to live long healthy lives.

Related Terms

Autosomal dominant, dermatopathia pigmentosa reticularis, ectodermal dysplasia, hyperpigmentation, hypohidrosis, incontinentia pigmenti, Nageli-Franceschetti-Jadassohn dysplasia, palmoplantar keratoderma, reticulate pigmentary disorders.