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Image challenge: Abdominal pain as the first manifestation of a systemic disease

What caused a symmetrical nonpruritic macular rash to develop on both feet of a 41-year-old patient?
Image Challenge
Image Challenge

Gastroenterology image challenge: A 41-year-old man without a significant previous medical history presented with a seven-day history of moderate upper abdominal pain, nausea and anorexia. On physical examination, the patient had left upper abdominal quadrant tenderness. Two days after admission, he developed a symmetrical nonpruritic macular rash on both feet, that evolved to palpable purpura and extended to both knees (figure A).

Laboratory investigation revealed neutrophilic leukocytosis (26,300/m3 white blood cells and 20,020/m3 neutrophils), elevated C-reactive protein (144 mg/L), hypoalbuminemia (2.5 g/dL) and folate deficiency (1.8 ng/mL). Urinalysis showed hematuria and nephrotic-range proteinuria. The immunologic profile was unremarkable. Viral infections were excluded and blood, urine and stool cultures were negative.

A contrast-enhanced computed tomography scan showed a 10-cm-long asymmetric proximal jejunal wall thickening with mural stratification and multiple lymph node enlargements (figure B). Esophagogastroduodenoscopy showed diffuse mucosal congestion and edema along duodenum with multiple erosions and deep ulcers starting in the bulb–D2 transition and worsening distally (figure C, D). Biopsies were taken and histologic examination revealed inflammatory infiltrate with polymorphonuclears.

What might be the diagnosis and how should it be managed?

To find out the diagnosis, read the full case in Gastroenterology.

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