We report 12 cases of Behçet's disease (BD) in children. The mean age of symptom onset was 12.4 years. Four patients (33.3%) had a past familial history of BD. Clinical manifestations were: oral aphtosis (n = 12), genital aphtosis (n = 9), ocular involvement (n = 9), neuro-Behçet (n = 6), venous thrombosis (n = 4), articular involvement (n = 3), and entero-Behçet (n = 1). All patients but one were initially treated with steroids; three cases with ocular involvement were treated with chlorambucil; and three other cases of neuro-Behçet were treated with cyclophosphamide. After a mean follow-up of 4 years, four patients with neurological involvement developed steroid-dependence with recurrence of symptoms. Four patients had optic atrophy with blindness.
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http://dx.doi.org/10.1080/03009740410005980 | DOI Listing |
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