Lymphedema, lymphocytic myocarditis, and sarcoidlike granulomatosis. Manifestations of Whipple's disease.
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.
Southern
J F
JF
Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston 02114.
Moscicki
R A
RA
Magro
C
C
Dickersin
G R
GR
Fallon
J T
JT
Bloch
K J
KJ
D000328:Adult; D003937:Diagnosis, Differential; D006099:Granuloma / Q000209:etiology*; D006801:Humans; D007421:Intestine, Small / Q000473:pathology; D008198:Lymph Nodes / Q000473:pathology; D008209:Lymphedema / Q000209:etiology*; D008264:Macrophages / Q000473:pathology; D008297:Male; D009205:Myocarditis / Q000188:drug therapy / Q000209:etiology*; D009206:Myocardium / Q000473:pathology; D011241:Prednisone / Q000627:therapeutic use; D012507:Sarcoidosis / Q000175:diagnosis; D008061:Whipple Disease / Q000150:complications / Q000175:diagnosis*
D011241:Prednisone