Mori M, Kuriyama T, Mitsuda T, Aihara Y, Yokota S, Chiba J, Nagaoka S
Department of Padiatrics, Yokohama-city University School of Medicine.
Ryumachi. 1994 Dec;34(6):988-92.
We reported a case of mother and child affected with Behçet's disease. Both had recurrent oral ulcer, erythema nodosum and arthralgia. Neither had gastrointestinal manifestation nor central nervous system involvement. The mother represented positive pathergy test, but the child did not. Laboratory data suggested no severe sign of inflammatory reaction and increased levels of immunoglobulin D. She was diagnosed as incomplete form, and he was diagnosed as suspicious form of Behçet's disease. On HLA examination, they had common haplotypes of HLA A24, B61, Cw1 and DR8. Additionally, haplotypes of HLA B7, Cw7, DR1, DQ1, were also detected in the mother, and HLA A2, Cw3, DR12, in the child. HLA-B51, which is primarily associated with Behçet's disease, was failed to be demonstrated. Familial involvement of Behçet's disease were described in only 20 cases, 11 of which had positive HLA B51. These findings suggest that molecular genetic examinations for both class I and II antigens will be necessary for a case of familial involvement.
我们报告了一例患有白塞病的母婴病例。两人均有复发性口腔溃疡、结节性红斑和关节痛。两人均无胃肠道表现及中枢神经系统受累。母亲针刺反应阳性,而孩子则为阴性。实验室数据显示无严重炎症反应迹象,免疫球蛋白D水平升高。她被诊断为不完全型,而他被诊断为白塞病可疑型。在HLA检查中,他们具有HLA A24、B61、Cw1和DR8的共同单倍型。此外,母亲还检测到HLA B7、Cw7、DR1、DQ1单倍型,孩子检测到HLA A2、Cw3、DR12单倍型。主要与白塞病相关的HLA-B51未能被检测到。白塞病的家族性受累仅在20例病例中被描述,其中11例HLA B51呈阳性。这些发现表明,对于家族性受累病例,对I类和II类抗原进行分子遗传学检查将是必要的。