慢性肉芽腫症について—難治性痔瘻で入院治療中に前頭骨骨髄炎を合併し症例

CHRONIC GRANULOMATOUS DISEASE, A CASE REPORT

　I．はじめに

　近年，感染防御機転に対する関心が深まり，いわゆる易感染性，あるいは反復感染をきたす疾患が注目を集めている。当科領域においても，反復性中耳炎や反復性上気道炎における免疫グロブリンの測定の結果についての報告が散見され，免疫不全症候群という用語も比較的ポピュラーなものとなつてきている。同様に易感染性の基礎疾患となる，食細胞側の機能不全については，症例がまだ少なく，今回報告する慢性肉芽腫症も本邦では最近5年間で37例を数えるにすぎないといわれ，耳鼻咽喉科関係の文献には皆無であつた。当症例もリンパ節炎，顔面の湿疹，顔面骨の骨髄炎など，日常診療において遭遇する症状を有し，この疾患の存在を念頭においてみたのではじめて発見されたもので，診断の確定が予後を左右する疾患であるので，ここに報告し，諸兄のご批判を仰ぎ，今後の診療の一助となれば幸いである。

　Chronic granulomatous disease is regarded as a syndrome of recurrent suppurative infection of the skin and reticuloendothelial organs associated with an inability of patients' phagocytes to kill bacteria which do not produce hydrogen peroxidase. The age of onset is usuall by one year.

　A case of fifteen month-old boy with this disease was presented.

　The first symptom of perianal abscess appeared by eight months of his life followed by lymphadenopathy and eczematous dermatitis near the external nares and external ears. The left forehead was struck by a falling book from the mother's hand and osteomyelitis with a fistula was developed.

　Surgery revealed a massive granuloma invading the lower part of the left frontal bone with a bony defect measured two centimeters in diameter and a centimeter in depth. Curretage of granulation tissue and circumscribed bone marrow and removal of the fistula yielded a good result.

　By negative NBT test which suggested failure of the patient's phagocytes to reduce nitroblue tetrazolium the diagnosis of chronic granulomatous disease was made.

　Though there were few reports of this disease in otolaryngology, each sign or symptom is popular in our field. Therefore the knowledge of and the interest to this disease should be broad-end in the pediatric otolaryngology.