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Our objective was to assess the increase in the number of children with Behçet's disease in France. To our knowledge, this survey is the most extensive reported from a single country.
Children with Behçet's disease from any part of France were referred to one of three medical centres: Marseille, Montpellier, and Paris. Information was obtained from the medical charts and from the patient's interview. A specific questionnaire was designed to determine the following demographic features: sex, age, city of residence, ethnicity, and familial history with complete pedigree; and the clinical variables: oral aphthous ulcers, genital ulceration, skin lesion, skin hypersensitivity plus other organ involvement—nervous system, gastrointestinal tract, eye, vessels, lungs, heart, joints, genitourinary tract, and fever. The date of onset of the disease was recorded together with the date of appearance of each symptom, and the date at which the patient met the international criteria for Behçet's disease.1 A specific database was set up.
Fifty five children with Behçet's disease met the international criteria before the age of 16 years: 33 white subjects (27 French), nine North Africans, five Turks, three West Indians, three mixed white/North African subjects, one Asian, and one Ashkenazi Jew. The male to female ratio was 0.89. The mean age of onset was 7.5 years (median 8 years, SD 4.3). The mean age at which patients met the criteria for Behçet's disease was 11.6 years (median 12, SD 3.7; fig 1). The mean time between the appearance of the first and last criterion was 3.5 years (median 3, SD 3.7).
Initial symptoms were oral ulcers in 41 (74%) (at a mean age of 6.8 years), genital ulcers in 13 (24%) (at a mean age of 6.8 years), bipolar aphthosis in nine (16%), skin lesions in eight (14%), and uveitis in two (4%). At least two criteria were present in nine (16%) patients.
Recurrent oral ulcers were present at a mean age of 7.44 years. Genital ulceration occurred in 43 (79%) patients, at a mean age of 10.8 years. Cutaneous signs included erythema nodosum (26%), necrotic folliculitis (38%), and aphthosis (14%). Ocular signs were uveitis (36%), retinal vasculitis (24%), conjunctivitis (17%), papilloedema (7%), and keratitis (3%). Arthralgia was the main articular sign, arthritis was present in 17% of patients. Headaches were common (35%) and associated with aseptic meningitis (10%), benign intracranial hypertension (10%), and hemiparesis in two patients. Abdominal pain was reported in 40% of cases, with digestive ulceration in 14%. Ulcerative colitis was diagnosed in one patient. Venous thrombosis occurred in 21% of patients. One 13 year old boy died of multiple deep vein thrombosis. Familial aggregation was present in four families which included eight patients (9%).
The epidemiology of paediatric Behçet's disease is difficult to evaluate because there is no formal agreement about the age of onset or the age of completed disease.2,3 Previous epidemiological studies have shown that the proportion of patients in whom the onset of symptoms occurs under the age of 16 years varies from 3% to 24%.4–7
Selection of patients according to international criteria gave high percentages of patients with mucocutaneous and ocular symptoms (reaching 60%, of which 36% were uveitis). The spread of clinical signs in our patients was similar to that obtained by other studies for patients of the same mean age and recruited similarly. The familial occurrence of BD, 9%, has been reported to be high in children and also in patients from endemic areas such as Turkey, Korea, and Tunisia.8–10 Therefore genetic linkage studies are needed to examine the genetic component of Behçet's disease further.
The number of recognised cases of Behçet's disease in children in France is increasing, probably reflecting an increase in doctors' awareness of this disease. Worldwide collaborations are now needed to delineate this subgroup of patients and to establish accurate sets of criteria.
Contributing doctors and nurses: Christine Bodemer, Brigitte Chabrol, Michel Cointin, Louis David, Paul Fisbach, Ariane Freychet, Catherine Glastre, Eric Grouteau, Jean Robert Harlé, Gilles Kaplanski, Iréne Lemelle, Alain Le Quellec, Josette Mancini, Anne Marie Prieur, Michéle Monticelli, Pierre Quartier dit Maire, Daniéle Sommelet, Jean Louis Stephan; The Association Behçet “B7” France and the Assistance Publique Hôpitaux de Marseille.