Preferred Label : Naegeli-Franceschetti-Jadassohn syndrome;
ICD-11 definition : Naegeli-Franceschetti-Jadassohn syndrome is a rare ectodermal dysplasia that affects
the skin, sweat glands, nails, and teeth. The cardinal features are absence of dermatoglyphics
(fingerprints), reticular cutaneous hyperpigmentation (starting at about the age of
2 years without a preceding inflammatory stage), hypohidrosis with diminished sweat
gland function and discomfort provoked by heat, nail dystrophy, tooth enamel defects,
and moderate hyperkeratosis of the palms and soles.;
Origin ID : 352035640;
Automatic exact mappings (from CISMeF team)
Naegeli-Franceschetti-Jadassohn syndrome is a rare ectodermal dysplasia that affects
the skin, sweat glands, nails, and teeth. The cardinal features are absence of dermatoglyphics
(fingerprints), reticular cutaneous hyperpigmentation (starting at about the age of
2 years without a preceding inflammatory stage), hypohidrosis with diminished sweat
gland function and discomfort provoked by heat, nail dystrophy, tooth enamel defects,
and moderate hyperkeratosis of the palms and soles.
