Koné-Paut I, Yurdakul S, Bahabri S A, Shafae N, Ozen S, Ozdogan H, Bernard J L
Department of Pediatrics, Hôpital Nord, Marseilles, France.
J Pediatr. 1998 Apr;132(4):721-5. doi: 10.1016/s0022-3476(98)70368-3.
The objective of this study was to characterize the clinical picture of Behçet's disease (BD) in children.
A questionnaire was completed by five BD specialists from Turkey, France, Iran, or Saudi Arabia. We first reviewed 86 cases retrospectively with a specially designed computerized database and then selected 65 who met the criteria of the International Study Group for BD, which include buccal aphthosis plus at least two among recurrent genital aphthosis, eye lesions, skin lesions, and positive pathergy test. The remaining 21 patients, who had features suggestive of BD but did not fulfill the international criteria, were analyzed separately and then compared with the other 65 patients.
BD affected boys and girls equally. The clinical picture frequently included mucocutaneous lesions. Uveitis was less frequent than in adults but carried a poor prognosis, especially in male patients (p < 0.001). The mortality rate (3%) was related to large vessel involvement. Familial cases were particularly frequent (15%). Erythema nodosum and skin hypersensitivity were common in Turkish patients, whereas neuro-BD was more frequent in French and Saudi Arabian patients. Patients who did not fulfill the international criteria had significantly less genital aphthosis (p < 0.01), less skin lesions or hypersensitivity (p < 0.01), and less uveitis (p < 0.01).
BD in children is similar to BD in adults. The high frequency of familial cases calls for further investigation of the immunogenetic factors that may favor early expression of the disease.
本研究的目的是描述儿童白塞病（BD）的临床特征。
来自土耳其、法国、伊朗或沙特阿拉伯的五位BD专家完成了一份问卷。我们首先使用专门设计的计算机数据库对86例病例进行回顾性分析，然后选择了65例符合BD国际研究组标准的患者，这些标准包括口腔阿弗他溃疡加复发性生殖器阿弗他溃疡、眼部病变、皮肤病变和针刺反应阳性中的至少两项。其余21例有BD提示特征但未达到国际标准的患者进行单独分析，然后与其他65例患者进行比较。
BD对男孩和女孩的影响相同。临床特征常包括黏膜皮肤病变。葡萄膜炎的发生率低于成人，但预后较差，尤其是男性患者（p<0.001）。死亡率（3%）与大血管受累有关。家族性病例尤为常见（15%）。结节性红斑和皮肤超敏反应在土耳其患者中常见，而神经白塞病在法国和沙特阿拉伯患者中更常见。未达到国际标准的患者生殖器阿弗他溃疡明显较少（p<0.01），皮肤病变或超敏反应较少（p<0.01），葡萄膜炎较少（p<0.01）。
儿童BD与成人BD相似。家族性病例的高发生率要求进一步研究可能有利于该疾病早期表现的免疫遗传因素。
