[A case of Behçet's disease in a child: clinical, genetic, and immunologic characteristics]

Behçet's disease, which is rarely observed in infancy, is a systemic inflammatory disease of unknown etiology, whose clinical feature consist in the triad of mouth ulcers, genital ulcers and iritis. A 14 years old girl has been studied in whom classical findings of Behøcet's disease were associated with neurological symptoms miming a neuro-Behøcet's syndrome, but which were caused by previous abuse in corticosteroid therapy. Interestingly some features (arthritis, gastrointestinal manifestations), which can be connected with Behøcet's disease, resulted to be present since the very first years of life. Partial features of the disease (arthritis or mouth ulcers) were present in three members of the patient's family on the father's side. Extensive immunological studies have been carried out. T and B lymphocyte number and function were normal. T-cell subsets (defined by monoclonal antibodies) and natural killer activity (both never examined in patients with Behøcet's disease) resulted to be within normal range. Secretory Component, which has been claimed to be absent in these patients, was normally present in saliva. A defect in neutrophil chemotaxis has been found which promptly improved by levamisole therapy. Authors discuss clinical, genetic and immunological findings of the patient, on the basis of a review of literature.