ABSTRACT
Mal de Meleda is a recessive transgressive palmoplantar keratoderma. We report the epidemiological, clinical and progressiveness aspects of the disease in 16 patients of a large consanguineous Tunisian family, the age ranged from 3 to 90 years. Nine were male and 7 female patients. Onset in early infancy was characterized by erythema of the palms and sole, followed by a diffuse yellowish hyperkeratosis. Thickness of the dorsal aspect of the fingers started in childhood. Extension of hyperkeratosis to the dorsa of the hand were reported only in adults and usually precede the involvement of the feet Palmoplanatar hyperhidrosis with fetid odor between the toes was frequently seen as well as conical distal phalanges. In some adults, keratoderma gave contracture of the fingers leading to limitation of movementsMal de Meleda must be differentiated from other recessive plamoplantar keratodermas such as Papillon le fever syndrome and male de Naxos the remaining inherited pal moplantar keratodermas being autosomal dominant. Mal de meleda is rare disease but is still relatively frequent in some Tunisian regions, due to the high rare of consanguinity Mal de Meleda remains an afflicting and disabling disease