[Digestive and nutritional manifestations of Netherton's syndrome]

Netherton's syndrome is a group of skin diseases where the cutaneous lesions may be associated with chronic digestive symptoms. We have observed five cases over a 10 year period, of which three in the same family. Management of nutritional and digestive problems was of major importance for vital prognosis. The literature data concerning the digestive involvement in Netherton's syndrome and the different associations between skin diseases and gastro-intestinal signs have been reviewed. The presence of villous atrophy in three of the five children suggests that a jejunal biopsy should be carried out in each case of Netherton's syndrome. Nutritional artificial support appears primordial at the initial stage of the illness, in order to maintain normal growth. Allergenic protein-free diet should not be systematic but discussed for each case and depend on the allergic and digestive laboratory results.