Capsule endoscopy in the management of a patient with a rare syndrome

Clinical presentation

A 54 year old man presented with bilateral lower limb oedema and pleural effusions, hypoalbuminaemia (18–22 g/l), and slowly growing yellow nails on both hands and feet (fig 1). Faecal content of α₁ antitrypsin and fat were increased, indicating protein loosing enteropathy. H₂ breathing test did not support bacterial overgrowth in the small intestine whereas a SeHCAT test indicated malabsorption of bile acids. Barium enteroclysis revealed altered bowel wall structure compatible with lymphoid hyperplasia but no signs of intestinal inflammation. Normal lymph flow and no signs of lymphatic obstruction in the lower extremities were documented with lymphoscintigraphy. Upper gastrointestinal endoscopy was normal.

Figure 1 Dystrophic yellow nails with ridging and loss of lunula on the hands.

Question

What is the diagnosis?

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