- Whipple's intestinal lipodystrophy
- Whipple's syndrome
Idiopathic steatorrhea, intestinal lipodystrophy, intestinal lipogranulomatosis, lipodystrophia intestinalis, lipophagic intestinal granulomatosis, mesenteric chyledenectasis, nontropical sprue, steatorrhea arthropericarditica.
A rare disorder resembling idiopathic steatorrhea. It is characterized by abnormal skin pigmentation, malabsorption, fatty stools, rapid wasting with loss of weight and strength, chills fever, chronic arthritis with joint pain, a distinctive lesion of the mucosa of the jejunum and ileum. Other features include aortic valve abnormalities and prominent lymph nodes.
This is a systemic disease caused by infection with a bacterium which was named Tropheryma whippelli. In the past, the disease was predominant in males, with onset usually between the ages of 30 and 50. It is now rare and is almost always curable.
Whipple in 1907 described a medical missionary with a condition "characterized by a gradual loss of weight and strength, stools consisting chiefly of neutral fat and fatty acids, indefinite abdominal signs, and a peculiar multiple arthritis". In addition, the patient had low blood pressure, skin pigmentation and anaemia.
The disease picture reported by Whipple had been described as early as in 1895 by Sir William Henry Allchin (1846-1922) and Richard Grainger Hebb (1848-1918).
Whipple's autopsy report:
Aot 36 years. Ward C,2. Autopsy No. 2883.
Died 10.40 P. M. May 8, 1907.
Autopsy 2 P. M. May 9, 1907.
Anatomical Diagnosis: Neutral fat and fatty acid deposits in intestinal mucosa - mesenteric and retroperitoneal glands and thoracic duct; chronic lymphadenitis; annemia; emaciation; organizing peritonitis, pleuritis, pericarditis and aortic endocarditis; cardiac dilatation and hypertrophy with fatty degeneration; chronic passive congestion of viscera; splenic tumor; hyperplasia of bonemarrow; cloudy swelling of viscera; leparotomy wound; bronchopneumonia and oedema of lungs; caseous apical scar and tuberculous bronchial lymphadenitis.
- W. H. Allchin and R. G. Hebb:
Transactions of the Pathologic Society of London, 1895, 46: 221-223.
- G. H. Whipple:
A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues.
Bulletin of the Johns Hopkins Hospital, 1907, 18: 382-391.
- A. D. Morgan:
The first reported case of Whipple's disease? Gut, 1961, 2: 370-372.