Weber-Christian病における免疫学的検討

WEBER-CHRISTIAN'S DISEASE WITH CELLULAR IMMUNO-DEFICIENCY

　Weber-Christian病（以下W-Chr．病と略す）の本態は明らかでないが，近年その病因論において膵疾患との関連^1〜3），脾の小動脈におけるonion skin lesionの発見^4），腎糸球体におけるhematoxylin body, wire loop様変化^5），リポイドネフローゼ様変化^6,7）から本症は膠原病として考えられて来ており，臨床的にリウマチ^8），全身エリテマトーデス^9），皮膚筋炎^10），鞏皮症^4），多発性血管炎^11）などとの合併が報告されている．また，本症患者に慢性細菌感染症を伴うものがあり，その多くがむし歯^12〜16），慢性扁桃炎，中耳炎，虫垂炎で，さらには結核の合併^17）も報告されている。組織学的には血管炎，血管壁のフィブリノイド変性の報告が多く，飯島^18），佐野^19），佐藤^20）らはその原因を感染アレルギーに求めている．

　著者らはこれらの観点からW-Chr．病の1例について2，3の免疫学的検索を行ない，細胞性免疫不全状態の存在を推定し得たので，文献的考察とともに自験例を報告する．

A case of this disease in a 25-year-old woman was reported. She had erythema nodosum-like eruptions on the trunk and extremities, the histologic pictures of which showed the panniculitis mainly composed of lymphoreticular infiltration and necrotizing angitis.

Hematologic studies of the peripheral blood showed marked anemia, leukopenia, especially lympho-penia. Studies on bonemarrow showed mildly decreased lymphocytic, inhibited maturation of granulo-cytic, and predominance of erythroblastic lines.

She was liable to suffer from the bacterial and viral infections such as bronchitis, pneumonia and herpes zoster etc. throughout the course of the disease.

Increase of her serum immunoglobulin level, slight decrease of serum complement level, marked de-crease of lymphocyte count in the blood, rather low degree of percentage of lymphocytes transformed on culture with PHA, and conversion of tuberculin reaction from positive to negative were proved. She could not be sensitized by DNCB.

Marked atrophy of lymphfollicles of the lymphnode and spleen has been proved in the literature. These evidences and results of the authors' clinico-laboratory studies strongly suggested deficiency state of cellular immunity in this disease.