J Rheum Dis.  2012 Jun;19(3):147-151. 10.4078/jrd.2012.19.3.147.

A Case of Intestinal Behcet's Disease Complicated Enterocutanous Fistula with a Good Response to Adalimumab

• Oh IH
• Kim JO
• Byun YJ
• Sung YK
• Choi CB
• Kim TH
• Jun JB
• Uhm WS

• KMID: 2223096
• DOI: http://doi.org/10.4078/jrd.2012.19.3.147

Abstract

Behcet's disease (BD) is a multisystemic disorder, which is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesion. Although intestinal involvement is an uncommon manifestation of the BD, it leads to a poor prognosis, as a result of a high complication, such as intestinal perforation, fistula formation, and massive hemorrhage. Intestinal BD has the tendency for the resistance to conventional medical treatment, and it often requires a surgical intervention because of severe complication. Although the causes of intestinal BD are unknown, some studies show that tumor necrosis factor Alpha (TNF-alpha) plays a key role in the pathogenesis of BD. Therefore, anti-TNF-alpha monoclonal antibody, such as adalimumab, is one of the useful treatment for refractory and relapsed intestinal BD. We describe a patient who had intestinal BD complicated enterocutanous fistula with a good response to adalimumab.

Keyword

Behcet's disease; Intestine; TNF-alpha; Adalimumab

MeSH Terms

Antibodies, Monoclonal, Humanized
Fistula
Hemorrhage
Humans
Intestinal Perforation
Intestines
Prognosis
Skin
Stomatitis, Aphthous
Tumor Necrosis Factor-alpha
Ulcer
Uveitis
Adalimumab
Antibodies, Monoclonal, Humanized
Tumor Necrosis Factor-alpha

Figure

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  Figure 1 Skin biopsy shows mononuclear cell vasulitis with nuclear dusts and fibrioid necrosis (H&E, ×400).

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  Figure 2 Initial colonoscopy shows well demarcated large ulcers with a clear ulcer bed at proximal ascending colon site (A), and three month after the first adalimumab injection, follow up colonoscopy shows improvement of previous ulcers (B).

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  Figure 3 Colonic Biopsy shows infiltration of chronic inflammatory cell with predominant neutrophils and granulation tissue (H&E, ×400).

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  Figure 4 One month (A) after medical treatment of steroid and azathioprine and three month (B) after first administration of adalimumab, abdomen computed tomography scan shows subcutaneous abscess (arrow) with enterocutaneous fistula formation and improvement of lesions (head).

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