頻回のフルニエ壊疽を繰り返した骨髄異形成症候群に合併した腸管 Behcet 病の1例 [in Japanese] A Case of Intestinal Behcet Disease and Myelodysplastic Syndrome Complicated by Fournier's Gangrene [in Japanese]
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症例は48歳,男性.高熱ならびに全身の紅斑を主訴に当院受診入院となった.陰嚢の腫大,潰瘍,蜂窩織炎(フルニエ壊疽)を指摘され,陰嚢切開にて軽快退院となった,貧血が持続するため精査施行し,骨髄異形成症候群(MDS, RA type)と診断された.フルニエ壊疽再燃加療中,腸管穿孔をきたし緊急手術となった.回腸に多数の穿孔部を認め回盲部切除をした.術後,消化管精査にて,大腸小腸に多発するpunched out ulcerならびに散在するアフタ性病変を指摘された.口腔内にもアフタ性病変指摘され,腸管Behcet病と診断された.在宅高カロリー輸液に移行し現在も加療中である.<BR>フルニエ壊疽の原因は,MDSによる易感染性と腸管Behcet病による陰部潰瘍によるものと推定された.MDSとBehcet病の合併例は本邦報告36例であった.
A 48-year-old man visited our hospital complaining of high fever and systemic eruption. With swelling, redness and ulcerations of the scrotum, he was diagnosed as Fournier's gangrene and treated by incision and drainage. He had been occasionally treated in the same way for Fournier's gangrene. As severe anemia was pointed out, further examination for the cause of anemia was done and he was diagnosed as myelodysplastic syndrome (MDS RA type).<BR>He was readmitted for therapy of recurrent Fournier's gangrene. After incision of the scrotum and castration, complication with panperitonitis due to perforation of the gastrointestinal tract was found. Multiple small punched-out ulcerations at the small intestine were revealed and ileocecal resection was performed. Postoperative colonoscopy revealed intestinal Behçet disease for multiple ulcerations and scars at the small and large intestine. He has been receiving home parenteral nutrition. Here we summarize 36 cases of Behçet disease associated with MDS in Japan.
- The Japanese journal of proctology
The Japanese journal of proctology 59(7), 385-389, 2006-07-01
The Japan Society of Coloproctology