Behcet's disease in children.

Abstract: Behcet's disease (BD) is not rare in children, and it's clini­cal picture differs from that in adults. We studied 66 patients in whom the disease was diagnosed under the age 16 from a population of 2068 BD patients. Comparison of the results with the adults by the student paired t test showed: although the most frequent initial manifestation of BD in children was oral aphthosis (54.5%), it was less frequent than in adults (73%. P < 0.0008). Uveitis as the initial manifestation was more frequent (30% vs 12%, P = 0.00002). Mucosal and skin manifestations of the disease were seen less in childhood (oral aphthosis: 77% vs 96%, genital aphthosis: 26% vs 65%, both with P< 0.000001, skin lesions: 61% vs 78%, P<0.0006). Ophthalmological lesions were more prevalent as anterior uveitis (67% vs 51 %, P< 0.02) and posterior uveitis (68% vs 50%, P< 0.004). Joint involvement in child­ren presented itself mostly as arthralgia. Arthritis was less encountered (14% vs 33%, P < 0.0005). We did not see any form of vascular involvement in children. HLA typing showed a higher frequency of HLA-B27 (21% vs 10%, P < 0.002). The complete form of the disease (Japanese Criteria) was less in children (6% vs 32%, P < 0.000001). To find if these differences will remain after the age of 16, we studied a group of 296 patients in whom the first manifesta­tion of the disease had begun before the age 16. It was notable that genital ulcerations (55% vs 65%, P < 0.002), phlebitis (6% vs 10%, P < 0.02) and complete form of the disease (25% vs 32%, P < 0.04) would be less encountered in these group, even in the long term follow-up (mean 11.9 years). Another important point is that the International Criteria is not suitable for diagnosing BD in children because of man­datory oral aphthosis. This cause a delay in diagnosis (only about half of the children have oral aphthosis as the initial manifestation), while having a low sensitivity (23% of the children never developed oral aphthosis before 16).