Naegeli-Franceschetti-Jadassohn syndrome: A rare case

Abstract

Naegeli-Franceschetti-Jadassohn Syndrome (NFJS) is a rare, autosomal dominant inherited form of ectodermal dysplasia, caused by mutation in the KRT14 gene. We report here a case of NFJS in a 27-year-old male who presented with reticulate hyperpigmentation over skin, dental changes, absence of dermatoglyphics, hypohidrosis, and hair changes.

Keywords

• Dermatopathia pigmentosa reticularis
• KRT 14 gene
• Naegeli-Franceschetti-Jadassohn
• reticulate hyperpigmentation