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Whipple Disease

Christos St. Basagiannis, MD, George S. Panagoulias, MD, Nicholas Tentolouris, MD, PhD, Stavros Basoukeas, MD, Dimitrios Sambaziotis, MD, Spiros D. Ladas, MD, PhD
Volume: 103 Issue: 4 April, 2010

Abstract:

Whipple disease (WD) is a rare disease caused by Tropheryma whipplei. The classic profile of the patient is that of a middle-aged man presenting with fever, chronic diarrhea, and arthralgias. Extragastrointestinal manifestations are not rare. A high degree of clinical suspicion for the disease is needed in atypical cases. Trimethoprim-sulfamethoxazole is the treatment of choice. We present two patients with WD. The first presented with melena and generalized hyperpigmentation. The second had depression for two years before the typical symptoms. Both hyperpigmentation and long-lasting depression without the typical manifestations of the disease are rare. Histologic examination of tissue biopsies was diagnostic for WD. Both patients were treated successfully with trimethoprim-sulfamethoxazole.


Key Points


* The classic profile of a patient with Whipple disease is that of a middle-aged man presenting with fever, chronic diarrhea, arthralgias, and fever; central nervous system involvement and psychiatric manifestations are not rare.


* Trimethoprim-sulfamethoxazole is the treatment of choice.


* Clinical improvement is the best criterion of response.


* The follow up of the patients should be performed at 6 and 12 months after initiation of treatment and then every year for a total of 3 years.

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