A case of Beh double dagger et's disease with widespread perforating enteric ulcers preceded by a long history of peripheral gangrene

作者：Honne Kyoko; Kohsaka Hitoshi^*; Kaneko Hideki; Komano Yukiko; Nakanishi Shoichi; Kitagawa Masanobu; Miyasaka Nobuyuki

来源：Modern Rheumatology, 2011, 21(6): 651-654.

DOI：10.1007/s10165-011-0444-5

• Behcet's disease
• Gangrene of the extremities
• Intestinal Behcet's disease

Patients suffering from Beh double dagger et'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 Beh double dagger et'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.

• 出版日期2011-12