Vol.21 No.6

Case Report

A case of Behçet’s disease with widespread perforating enteric ulcers preceded by a long history of peripheral gangrene

Authors

Kyoko Honne1 , Hitoshi Kohsaka1 , Hideki Kaneko1 , Yukiko Komano1 , Shoichi Nakanishi2,3 , Masanobu Kitagawa2 , Nobuyuki Miyasaka1

  • Department of Medicine and Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
  • Department of Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
  • Department of Oral Pathology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Received:

14 January 2011

Accepted:

28 February 2011

Published online:

30 March 2011

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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.

Key words

Behçet’s disease - Gangrene of the extremities - Intestinal Behçet’s disease