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报告2例尸检及1例活检白塞氏病,属于不完全型,青年女性(尸检)及青年男性(活检)。以口腔及外阴部反复性溃疡及皮肤病变为主。皮肤针反应呈脓疱状。口腔粘膜及外阴部溃疡处乳头内小血管基底膜增厚,IgM、IgA、IgG沉着。全身小血管均见内皮细胞肿大,管腔狭小,管壁基底膜增厚。小动脉尚有平滑肌细胞肥大,数量增多。主动脉虽肉眼未见改变,中膜粘液蓄积,弹力纤维紊乱,外膜自养血管的小动脉管壁厚有IgA沉着。小静脉周炎细胞浸润,肉芽肿形成。大静脉内膜见阶段状内膜缺损,未见文献有相似记载。
Two cases of autopsy and one case of biopsy Behcet’s disease were reported, belonging to incomplete type, young women (autopsy) and young men (biopsy). Repeated ulcers and skin lesions in the mouth and vulva are the major causes. The skin reaction was pustular. The basement membrane of the small blood vessels in the nipple of the oral mucosa and the vulva ulcers was thickened, and the IgM, IgA, and IgG were calm. Small blood vessels in the whole body are seen with enlargement of endothelial cells, narrow lumens, and thickened basement membrane. There are still smooth muscle cells in the small arteries with hypertrophy and increased numbers. Although the aorta was not changed by the naked eye, mucus accumulated in the media, the elastic fiber was disordered, and the thickness of the arterioles in the adventitia of the adventitia was IgA. Infiltration of small perivenous cells, granuloma formation. The venous intima was seen in the intimal defect of the great vein, and no similar literature was recorded in the literature.