多彩な症状を呈したBehçet病の1剖検例

BEHCET'S DISEASE WITH DIVERSE MANIFESTATIONS

はじめに

　Behçet病^1）2）が3主徴（アフタ・陰部潰瘍・前房蓄膿を伴う再発性葡萄膜炎）以外に，他臓器に障害をきたすことはすでに諸家^3〜7）によつて報告されている。すなわちNeuro-Behçet^{8）9）10）}，Intestinal Behçet^11），Cardio-Behçet^12）やAngio-Behçet^13）3）等の名で呼ばれる，中枢神経系・消化器系・循環器系・血管系の侵襲をみても，本症の症状がいかに多様性を示すものであるかを物語つて余りある。

　皮膚における発疹も上記3主徴に優るとも劣らぬ発現がみられる所から，3主徴に皮膚を加えて4主徴とする考えがあり，皮疹は結節性紅斑型皮疹・血栓性静脈炎型皮疹および毛嚢炎ないし痤瘡様皮疹の3型またはその混合型で示される^14）。上述したこれら多くの症状は再燃と寛解の反復を慢性遷延性にくり返し^4），しかも大半が進行性の経過をとつて致命あるいは失明の終末を辿つている。

　A 38-year-old business man suffered from erythema nodosum-like nodules on the extremities, aseptic pustules, aphthae and fever over 40℃ after the end of 1961. He visited the author's clinic at first in February, 1962 and was under the author's control. In November, 1962 he complained of pain in the ileocaecal region, which was due to perforation of the ileum and adhesion between the jejunum and the vermiform appendix. Seventeen days after the operation radial pulse on either side could not be palpable alternatively. From that time, several attacks of such a loss of radial pulse were proved at the periods of recurrence. In November, 1963, a sudden attack of right hemiplegia, loss of muscle strength of the right half of the body, hiccup and the abnormal articulation of words were noticed. In August, 1967 a stone in the left kidney was found.

　He was admitted to the hospital because of hypochondrial pain on February 7, 1967. Leukocytosis, increased ASLO titer and severe disturbance of liver functions were proved. He died of coma.

　Autopsy revealed acute yellow liver atrophy, and demyelinization in the brain and spinal cord.