An Unusual Case of Whippleʼs Disease Discovered During Screening Endoscopy

Abstract

Introduction: Whipple's Disease is a rare disease that results in a chronic systemic infection. The bacterial culprit responsible is a gram positive bacillus known as Tropheryma whipplei. The classic initial presentation is a constellation of gastrointestinal symptoms of diarrhea, weight loss, and abdominal pain; however, not all cases present with such findings. Despite a multitude of advances in diagnostic fields, Whipple's Disease remains elusive and difficult to diagnose. We present a rare case of Whipple's Disease in a patient undergoing screening for esophageal varices. Case: We present a case of a 77 year old Caucasian male with past medical history of diabetes, hypertension, and alcoholic cirrhosis. The diagnosis of alcoholic cirrhosis was made approximately 5 years ago at a different institution. During his initial office visit, he reported no complaints, except for nonspecific chronic arthralgia. The patient had not undergone screening for esophageal varices, and was scheduled for endoscopy in an outpatient setting. The EGD revealed a Schatzki's ring, pale friable eroded mucosa in the distal esophagus, a 3mm polyp in the gastric body (Figure 1), and an 8mm eroded polyp in the gastric cardia. The gastric cardia polyp and distal esophageal mucosa were biopsied and results were unremarkable. The 3mm gastric body polyp demonstrated gastric mucosa with numerous PAS positive histiocytes (Figure 2) in the lamina propria containing vacuoles, which in turn contained bacteria undergoing lysis according to electron microscopy exam (Figure 3). These findings were strongly suggestive of Whipple's Disease. As his only symptom was non-specific chronic arthralgia, treatment for Whipple's Disease was deferred. Plans are made for follow-up EGD to evaluate and take random biopsies of the small bowel for pathologic examination.Figure: Body of stomach lesion where Whipple's was found.Figure: PAS-D eosinophilic structures within the histiocytes.Figure: A) Numerous phagocytized bacteria within lysosomes B) Intact bacterium with recognizable cell wall present within the cytoplasm of the histiocyte.Discussion:Tropheryma whipplei is a bacterium known to cause a rare illness in humans, Whipple's Disease. This disease typically involves multiple organ systems, including but not limited to the gastrointestinal tract, joints, and central nervous system. A brief review of the literature suggests that there are only few cases of Whipple's Disease diagnosed each year. Our case describes a patient presenting for screening of esophageal varices found to have Whipple's Disease based off biopsy of a gastric polyp. Endoscopic visualization may be helpful in considering diagnosis in the future.