RETICULAR PIGMENTED GENODERMATOSIS WITH MILIA - A SPECIAL FORM OF NAEGELI-FRANCESCHETTI-JADASSOHN SYNDROME OR A NEW ENTITY

A 19-year-old woman with a condition since birth, comprising reticular hyperpigmentation, palmoplantar hyperkeratosis, dental anomalies, hyp oplasia of dermatoglyphics and a slight yellowish hue to the nails is presented. Her father and grandfather were similarly affected. The ret icular hyperpigmentation, although generalized, was more intense on th e flexural areas where extensive milia formation was also observed. Th is case represents an unusual genodermatosis. The milia formation and the presence of normal perspiration indicate a special form of Naegeli -Franceschetti-Jadassohn (NFJ) syndrome, or a new entity close to the NFJ syndrome. The differential diagnosis from other congenital reticul ate pigmentary disorders is discussed.