Degos病の1例―本邦7症例の集計を含めて

Degos' Disease: Report of a Case and a Review of Seven Cases in Japan

　Degos病（malignant atrophic papulosis，悪性萎縮性丘疹症）は主として皮膚および消化管に発生するまれな疾患であり，1948年にフランスの皮膚科医Degosらによって命名されたことよりその名がある．本症はその初発症状が特有な皮疹であるためか，従来より主として皮膚科の領域で報告，検討がなされてきた．しかし，本症は高頻度に消化管障害を来し穿孔により死亡する極めて予後不良な疾患である．今回，われわれはこのDegos病の典型的な1例を経験したので報告すると共に，本邦報告例を集計し検討した．

　The patient was a 44 year-old man who noticed red papules scattered over the whole body skin about two years before admission. Thereafter, the patient occasionally complained of lower abdominal pain, and about nine months prior to admission, abdominal operation was performed because of acute diffuse peritonitis due to intestinal perforation. This time he was referred to our hospital under the diagnosis of acute diffuse peritonitis. Though partial small intestinal resection and simple closure of the perforated intestine were performed, he died on the twelfth postoperative day. At autopsy, multiple minute ulcers and perforations were found diffusely on the small intestine. The same lesions were also found in the stomach, large intestine, and urinary bladder. Microscopic examination showed obliterative endovasculitis, namely, thickening and cellular proliferation of intima with luminal narrowing and obstruction in small arteries and veins.

　Only seven cases including this patient have been reported in Japan. In all cases, skin lesions were the initial symptom and four cases died of acute diffuse peritonitis. As no effective treatment has been established for this disease, the clinical prognosis seems to be very poor.