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Tuesday, March 8, 2011


This is rare disease is important because it is curable. There is characteristically infiltration of the intestinal mucosa and other organs with macrophages which stain positively with periodic acid-schiff (PAS) stain.
Electron-microscopy shows numerous bacilliform bodies indicating that the disease is probably caused by an organism which has yet to be defined.

Clinical features
The patient presents with malabsorption and steatorrhoea consequent upon the infiltration of macrophages which interfere with lymph flow and thus restrict the absorption of nutrients. There is polyarthritis, fever, weight loss, abdominal pain and respiratory symptoms. On examination lymphadenopathy, anthropathy, skin pigmentation and abdominal distension and tenderness may be defined.
The diagnosis is made by jejunal biopsy.

Treatment consists of antibiotic for 1 year, tetracycline 1g per day being the drug of choice. Relapses occur and the patient must be followed-up permanently.

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