Baylor University Medical Center Proceedings October 2017 - 429

An unusual presentation of Tropheryma whipplei infection
Trace Heavener, DO, Michelle Thompson, DO, Chhaya Patel, MD, Lisa Forrester, MD, PhD, and Donald Rawls, MD

Whipple's disease is an infection caused
by the Gram-positive bacillus Tropheryma
whipplei. Invasion or uptake of the bacteria can occur in various parts of the body.
The differential diagnoses are broad due
to the wide spectrum of infection, and the
disease is diagnosed based on biopsy of
suspected lesions, usually in the small intestine. We present the case of a 56-yearold man with no significant prior medical
history who presented with swelling and
pain in the left eye. Review of systems
revealed 6 months of persistent diarrhea, Figure 1. Small bowel biopsy showing (a) blunted villi and expansion with periodic acid-Schiff-positive macrophages
and intestinal biopsy revealed periodic (arrow) and lipid droplets (star) (100×); and (b) mucin in goblet cells (black arrow) and macrophages with periodic
acid-Schiff-resistant material (white arrow) (400×).
acid-Schiff-positive macrophages.

hipple's disease was identiﬁed in the early 1900s, but
it was not cultured until 1997 (1). It is caused by
Tropheryma whipplei, a Gram-positive bacillus that
is related to actinomycetes and is fairly ubiquitous
in nature with a wide spectrum for infection, including the
intestinal epithelium, macrophages, capillary and lymphatic
endothelium, colon, liver, brain, heart, lung, synovium, kidney,
bone marrow, and skin (2). Due to the infection's widespread
involvement, clinical manifestations are broad but most commonly include arthralgia, weight loss, diarrhea, and abdominal pain. The incidence of Whipple's disease is low; this case
featured an unusual presentation.

CASE REPORT
A 56-year-old man with no signiﬁcant prior medical history presented with swelling and pain in the left eye. He was
found to have a complicated pseudomonal infection involving
the periorbit and sinuses as well as bilateral pneumonia with
cavitary disease. For 6 months, he had endured persistent diarrhea, night sweats, fatigue, a 60-pound weight loss, and hyperpigmentation of the skin. Upper endoscopy showed gastritis
but no masses or bleeding ulcers. Small bowel biopsies were
obtained, and histologic evaluation demonstrated small intestinal mucosa with villous blunting and expansion with periodic
Proc (Bayl Univ Med Cent) 2017;30(4):429-430

acid-Schiﬀ-positive macrophages within the lamina propria
(Figure 1). Whipple's polymerase chain reaction (PCR) was
obtained but returned negative results. Treatment for Whipple's
disease was initiated with doxycycline and hydroxychloroquine.
The patient demonstrated complete remission of his symptoms.
DISCUSSION
The annual incidence of Whipple's disease is approximately
1 per 1,000,000 people (3). In general, it is noted to have two
distinct stages. The ﬁrst stage is a prodromal stage consisting of
nonspeciﬁc ﬁndings such as arthralgias, adenopathy, and fever.
The second stage is the steady-state stage consisting of organspeciﬁc ﬁndings such as diarrhea, weight loss, and neurological
symptoms. The typical time course from onset of the prodromal
stage to the steady-state stage is thought to be around 6 years,
but it can be triggered much more quickly in certain situations
such as an immunocompromised host (2).
The current methods for diagnosis are culture, serology,
pathology (i.e., duodenal biopsy), and molecular testing. Data
From the Department of Internal Medicine (Heavener, Patel, Forrester) and Division
of Gastroenterology (Thompson, Rawls), Baylor Scott and White Health, Temple,
Texas.
Corresponding author: Trace Heavener, DO, Baylor Scott & White Health, 2401 S.
31st Street, Temple, TX 76508 (e-mail: trace.heavener@bswhealth.org).
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