消化管病変を契機に診断した好酸球性多発血管炎性肉芽腫症の1例

Eosinophilic Granulomatosis with Polyangiitis Diagnosed Based on Digestive Tract Lesions, Report of a Case

要旨●患者は60歳代，男性．20XX−2年に気管支喘息を発症．20XX−1年に近医呼吸器内科で好酸球性肺炎と診断され，ステロイド内服加療中であった．20XX年に心窩部痛と血便が出現し当科を受診した．大腸内視鏡検査で遠位S状結腸〜直腸に周囲を縁取るような発赤を伴う地図状潰瘍の多発を認めた．カプセル小腸内視鏡検査では上部〜下部小腸にわたり大腸病変に相似する潰瘍性病変の散在を認めた．皮膚病変を検索したところ体幹と四肢に網状紫斑の多発を認め，臀部の網状紫斑より採取した皮膚生検組織で壊死性血管炎の所見を認め，好酸球性多発血管炎性肉芽腫症と確定診断した．心病変，神経炎の合併を認めシクロホスファミド大量静注療法を施行した．特徴的な小腸病変と大腸病変の内視鏡所見から本症を疑って皮膚病変を検索し，紫斑を皮膚生検した結果，本症の確定診断に至った症例であった．

　The patient was a man in his 60s. In 20XX-2 he developed bronchial asthma. In 20XX-1, he was diagnosed with eosinophilic pneumonia at the Respiratory Medicine Department of our hospital and received oral steroid treatment. In 20XX, the patient presented to our department with epigastric pain and bloody stools. A large intestine endoscopy revealed multiple geographical ulcers with redness surrounding the distal sigmoid colon and the rectum. A small intestine capsule endoscopy revealed scattered ulcerative lesions similar to colonic lesions in the upper and lower small intestine. A search for a skin lesions revealed multiple retiform purpura on the trunk and extremities, and a skin biopsy revealed necrotizing vasculitis in the tissue taken from the retiform purpura on the buttocks, leading to the definitive diagnosis of EGPA（eosinophilic granulomatosis with polyangiitis）. The patient was treated with high-dose intravenous cyclophosphamide because of cardiac lesions and neuritis. In this case, we looked for skin lesions because EGPA was suspected based on the endoscopic findings of characteristic small intestinal and colorectal lesions, and skin biopsy of the purpura led to the definitive diagnosis of EGPA.