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Manifestations of Whipple's Disease

James F. Southern, MD, PhD; Richard A. Moscicki, MD; Cynthia Magro, MD; G. Richard Dickersin, MD; John T. Fallon, MD, PhD; Kurt J. Bloch, MD

JAMA. 1989;261(10):1467-1470.

Abstract
A patient with Whipple's disease presented with a long prodromal period characterized by granulomatous lymphadenitis and progressive lymphedema of the extremities. No gastrointestinal tract symptoms were present and a small bowel biopsy sample was normal. His clinical condition deteriorated with the onset of lymphocytic myocarditis. At autopsy, intestinal involvement with macrophages that stained positively with periodic acid—Schiff was limited primarily to the submucosa. Diffuse fibrous effacement of lymph nodes with afferent lymphangiectasia seemed to be the mechanism of diffuse lymphedema, protein-losing enteropathy, and hypoproteinemia. Whipple's disease, therefore, should be considered in the differential diagnosis of patients presenting with granulomatous disease, lymphocytic myocarditis, or unusual lymphedema.

(JAMA 1989;261:1467-1470)

Author Affiliations
From the Departments of Pathology (Drs Southern, Dickersin, and Fallon) and Medicine (Drs Moscicki and Bloch), Harvard Medical School, and the Department of Pathology (Drs Southern, Magro, Dickersin, and Fallon) and the Clinical Immunology and Allergy Units, General Medical Services (Drs Moscicki and Bloch), Massachusetts General Hospital, Boston.

Footnotes
Reprint requests to Department of Pathology, Massachusetts General Hospital, Boston, MA 02114 (Dr Fallon).

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