title: A case of Behçet's disease with widespread perforating enteric ulcers preceded by a long history of peripheral gangrene.
abstract: Patients suffering from Behçet's disease (BD) with intestinal involvement often have an atypical disease course. The disease in many patients does not fully meet the diagnostic criteria defined by the International Study Group for Behçet's Disease, and instead such patients are diagnosed as having an incomplete type of BD according to the criteria of the BD Research Committee of Japan. The patient reported here developed uveitis and gangrene of the extremities as the initial symptoms and was treated with corticosteroid. After 16 years, the gangrene relapsed and multiple deep ulcers suddenly developed in the gastrointestinal tract, with oral ulcer and uveitis. The intestinal perforation was acute, progressive, severe, and extensive. With a diagnosis of incomplete BD with intestinal involvement, she was treated with high-dose glucocorticoid. However, uncontrollable gastrointestinal bleeding led to her death. Histopathological examination revealed that she suffered from intestinal and vascular BD, although gangrene of the extremities has rarely been reported as a manifestation of vascular BD. Thus, the disease course of this patient was characterized by the reappearance of peripheral gangrene after a long interval that preceded the devastating intestinal lesions.
authors: Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.
authors: Kyoko
authors: K
authors: Honne
authors: Hitoshi
authors: H
authors: Kohsaka
authors: Hideki
authors: H
authors: Kaneko
authors: Yukiko
authors: Y
authors: Komano
authors: Shoichi
authors: S
authors: Nakanishi
authors: Masanobu
authors: M
authors: Kitagawa
authors: Nobuyuki
authors: N
authors: Miyasaka
mesh_terms: D000368:Aged; D001528:Behcet Syndrome / Q000150:complications / Q000473:pathology*; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D005734:Gangrene / Q000150:complications / Q000473:pathology*; D006801:Humans; D007416:Intestinal Perforation / Q000150:complications / Q000473:pathology*; D013276:Stomach Ulcer / Q000150:complications / Q000473:pathology*; D014605:Uveitis / Q000150:complications / Q000473:pathology*