Clinical presentation
A 24 years old woman of Hong Kong origin but resident in the UK for the past 14 years presented with a four week history of abdominal pain, watery diarrhoea, and vomiting on a background history of adult onset Still's disease. She was found to be pyrexial with a raised erythrocyte sedimentation rate and an albumin of 25. Gastroscopy was normal but at colonoscopy she was found to have a solitary bleeding terminal ileal ulcer (fig 1).
Figure 1 Solitary bleeding terminal ileal ulcer.
Histology of the ulcer showed necrotising granuloma (fig 2). Following a presumptive diagnosis of Crohn's disease, she was started on 40 mg/day of oral prednisolone with an initial good clinical response but her symptoms returned along with large volume ascites four weeks after cessation of steroids. At this point she also developed a pancytopenia with a further fall in albumin(<16). Computed tomography of the chest and abdomen (fig 3) revealed small bilateral pleural effusions and small pericardial effusion with atelectasis of both lung bases along with large volume ascitis and multiple distended oedematous small as well as large bowel loops. Subsequent laparoscopy and peritoneal biopsies were normal.
Figure 2 Histology showing necrotising granuloma. G, granuloma with surrounding lymphocyte infiltration; L, Langhan's giant cell.
Figure 3 Computed tomography showing diffuse oedema of the bowel wall (target appearance).
Question
What is the diagnosis?
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