[A rare cause of neonatal exudative enteropathy: congenital Langerhans cell histiocytosis (histiocytosis X)].

A case of Langerhans cell histiocytosis is reported in a neonate. Intestinal involvement was especially diffuse and severe, presenting as a protein-losing enteropathy secondary to massive mucosal infiltration by histiocytic cells. The infant died at the age of 3 1/2 months despite therapy with corticosteroids and vinblastine then etoposide and interferon. Such an outcome confirmed the severity of forms with neonatal onset and digestive involvement.