Hyperpigmentation and hypohidrosis. (THE NAEGELI-FRANCESCHETTI-JADASSOHN SYNDROME): REPORT OF A FAMILY AND REVIEW OF THE LITERATURE

Seven individuals from one family are recorded who have a syndrome consisting of hyperpigmentation, hypohidrosis and dystrophy of the nails. Associated features are punctate keratoses of the palms and soles, hypoplasia of the dermatoglyphics, atrophic changes of the skin of the face, bullae on the feet and dental anomalies. The disorder is inherited as an autosomal dominant trait. The relationship between this and other syndromes with hyperpigmentation is discussed.